Pulsed Levosimendan in advanced heart failure due to congenital heart disease: a case series.

BACKGROUND: Levosimendan is a non-adrenergic calcium-sensitizing agent with positive inotropic and vasodilatory effects. Its use in acute decompensation of heart failure is established. Good evidence now exists for repetitive infusions of Levosimendan to improve symptoms and reduce hospitalization in advanced heart failure (AdHF) populations. Its use in heart failure resulting from congenital heart disease is not yet commonplace. CASE SUMMARY: We present three cases in which pulsed Levosimendan was used in the management of AdHF secondary to underlying congenital heart disease. There was symptomatic and biomarker evidence of improvement. DISCUSSION: Intermittent Levosimendan may represent a valuable therapy to reduce hospitalization and improve quality of life in adults with congenital heart conditions.

Maternal and neonatal outcomes in women with history of coronary artery disease.

BACKGROUND: Pregnancy outcomes in women with pre-existing coronary artery disease (CAD) are poorly described. There is a paucity of data therefore on which to base clinical management to counsel women, with regard to both maternal and neonatal outcomes. METHOD: We conducted a retrospective multicentre study of women with established CAD delivering at 16 UK specialised cardiac obstetric clinics. We included pregnancies of 24 weeks' gestation or more, delivered between January 1998 and October 2018. Data were collected on maternal cardiovascular, obstetric and neonatal events. RESULTS: 79 women who had 92 pregnancies (94 babies including two sets of twins) were identified. 35.9% had body mass index >30% and 24.3% were current smokers. 18/79 (22.8%) had prior diabetes, 27/79 (34.2%) had dyslipidaemia and 21/79 (26.2%) had hypertension. The underlying CAD was due to atherosclerosis in 52/79 (65.8%), spontaneous coronary artery dissection (SCAD) in 11/79 (13.9%), coronary artery spasm in 7/79 (8.9%) and thrombus in 9/79 (11.4%).There were six adverse cardiac events (6.6% event rate), one non-ST elevation myocardial infarction at 23 weeks' gestation, two SCAD recurrences (one at 26 weeks' gestation and one at 9 weeks' postpartum), one symptomatic deterioration in left ventricular function and two women with worsening angina. 14% of women developed pre-eclampsia, 25% delivered preterm and 25% of infants were born small for gestational age. CONCLUSION: Women with established CAD have relatively low rates of adverse cardiac events in pregnancy. Rates of adverse obstetric and neonatal events are greater, highlighting the importance of multidisciplinary care.

Maternal and fetal outcomes in pregnancies complicated by Marfan syndrome.

OBJECTIVES: Information to guide counselling and management for pregnancy in women with Marfan syndrome (MFS) is limited. We therefore conducted a UK multicentre study. METHODS: Retrospective observational study of women with MFS delivering between January 1998 and March 2018 in 12 UK centres reporting data on maternal and neonatal outcomes. RESULTS: In total, there were 258 pregnancies in 151 women with MFS (19 women had prior aortic root replacements), including 226 pregnancies ≥24 weeks (two sets of twins), 20 miscarriages and 12 pregnancy terminations. Excluding miscarriages and terminations, there were 221 live births in 139 women. Only 50% of women received preconception counselling. There were no deaths, but five women experienced aortic dissection (1.9%; one type A and four type B-one had a type B dissection at 12 weeks and subsequent termination of pregnancy). Five women required cardiac surgery postpartum. No predictors for aortic dissection could be identified. The babies of the 131 (65.8%) women taking beta-blockers were on average 316 g lighter (p<0.001). Caesarean section rates were high (50%), particularly in women with dilated aortic roots. In 55 women, echocardiographic aortic imaging was available prepregnancy and postpregnancy; there was a small but significant average increase in AoR size of 0.84 mm (Median follow-up 2.3 months) CONCLUSION: There were no maternal deaths, and the aortic dissection rate was 1.9% (mainly type B). There with no identifiable factors associated with aortic dissection in our cohort. Preconception counselling rates were low and need improvement. Aortic size measurements increased marginally following pregnancy.

Is a planned caesarean section in women with cardiac disease beneficial?

OBJECTIVES: In the general population, planned caesarean section is thought to be safer in high-risk situations as it avoids the greater risk of an emergency caesarean section. Only limited data exist on the optimal mode of delivery in women with structural heart disease. We investigated the relationship between mode of delivery and pregnancy outcome in women with pre-existing heart disease. METHODS: The Registry on Pregnancy and Cardiac Disease is an on-going, global, prospective observational registry of women with structural heart disease. We report on 1262 deliveries, between January 2007 and June 2011. RESULTS: The caesarean section was planned in 393 women (31%): 172 (44%) for cardiac and 221 (56%) for obstetric reasons of whom 53 delivered by emergency caesarean section. Vaginal delivery was planned in 869 (69%) women, of whom 726 (84%) actually delivered vaginally and 143 (16%) had an emergency caesarean section. Perinatal mortality(1.1 vs 2.7, p=0.14) and low apgar score (11.9 vs 10.1, p=0.45) were not significantly different in women who had a caesarean section or vaginal delivery; gestational age(37 vs 38 weeks p=0.003) and birth weight (3073 vs 2870 g p<0.001) were lower in women delivered by caesarean section compared with women delivered by vaginal delivery. In those delivered by elective or emergency caesarean section, there was no difference in maternal mortality (1.8% vs 1.5%, p=1.0), postpartum heart failure (8.8% vs 8.2% p=0.79) or haemorrhage (6.2% vs 5.1% p=0.61). CONCLUSIONS: These data suggest that planned caesarean section does not confer any advantage over planned vaginal delivery, in terms of maternal outcome, but is associated with an adverse fetal outcome.

Pregnancy and treatment outcome in a patient with left ventricular non-compaction.

Left ventricular non-compaction (LVNC) is a rare form of cardiomyopathy. This case reviews a woman with familial LVNC (EF 45%, NYHA class I, evidence of non-sustained ventricular tachycardia pre-pregnancy) who had significant decompensation with heart failure in the third trimester that required early delivery. Deterioration in symptoms and LV function 7 days after delivery required further hospitalization and aggressive treatment. Suppression of lactation with bromocriptine, together with standard heart failure management, has allowed recovery and return to full activities and work. Acknowledged adverse risk factors in LVNC are considered, and pre-pregnancy risk assessment is reviewed. There is no specific treatment for LVNC in pregnancy besides the usual management of dilated cardiomyopathy. This is the ninth case report of LVNC in pregnancy reported in the literature.

Infective endocarditis caused by Moraxella nonliquefaciens in a percutaneous aortic valve replacement.

First case of infective endocarditis on a percutaneous aortic valve replacement due to Moraxalla nonliquefaciens in 64 year old woman. It was successfully treated medically with antibiotics. She had not suitable for surgical aortic valve replacement due to 3 sternotomies for thymoma resection and subsequent radiotherapy with blocked major thoracic veins. Due to her azathioprine immunosuppresion (myasthenia) she may have been at increased endocarditis risk. We suggest prophylactic antibiotics at implant for this group in future.

Late complication of total anomalous pulmonary venous drainage in an adult: 'a diagnostic challenge'.

Total anomalous venous drainage is a rare congenital cardiac defect seen in 1% of the population; there is usually a concomitant atrial septal defect. We discuss such a diagnostic challenge in a patient who presented on a number of occasions with acute respiratory distress and haemoptysis. True understanding of her underlying congenital heart disease was not appreciated until review in a congenital heart disease clinic - in which she presented for prepregnancy counselling. She was found to have a stricture at the site of anastomosis of the common chamber with the left atrium, resulting in pulmonary venous hypertension leading to acute presentation of pulmonary haemorrhages. She underwent successful full balloon dilatation of the site, leading to full recovery. Most patients are diagnosed at birth and undergo surgical repair. The outcome is good, and historically it has been regarded that it is unusual for them to have long-term complications, although dysrhythmias remain a recognized long-term complication. Surgery for total anomalous pulmonary venous drainage is nevertheless a palliation, and it is vital that the possible role of late complications is considered in all cases.

Severe triple vessel coronary artery disease and aneurysms in a young white man: disease progression of childhood Kawasaki disease.

Kawasaki disease usually affects younger age groups, but cardiac sequelae of 'missed' (incomplete) childhood Kawasaki disease may first present in adult life. We report a case of a 28-year-old white man presenting with ST elevation myocardial infarction and later found to have triple vessel coronary artery aneurysmal disease, probably secondary to childhood Kawasaki disease for which he underwent cardiac bypass surgery. The patient has remained well and asymptomatic at 1-year follow-up. This case is highlighted as the incidence of Kawasaki disease is relatively low among the white population, and yet there are increasing reports of adult patients presenting with coronary artery aneurysmal disease secondary to childhood Kawasaki disease. There has been a relative paucity of reports of surgical revascularization for adult survivors of childhood Kawasaki disease. This report also emphasizes the importance of early recognition and aggressive treatment of the often missed, incomplete, form of Kawasaki disease, which may reduce the risk of late development of coronary artery aneurysms. There should be a high index of suspicion of Kawasaki disease in young adults presenting with aneurysmal coronary artery disease with no significant coronary risk factors and in the absence of generalized atherosclerosis.

Sarcoidosis, microvascular angina and aortitis: New dimensions of the 'Takayasu syndrome' - A case report.

A case of Takayasu aortitis associated with sarcoidosis presenting with recurrent angina is reported. This association has been called 'Takayasu syndrome', which reflects what is likely a shared etiology. Myocardial perfusion abnormalities have recently been documented in sarcoidosis, but this case clarifies for the first time that the angina in Takayasu syndrome is likely due to small vessel coronary arteritis. Corticosteroids and cytotoxic therapy have been shown to be beneficial in all forms of sarcoidosis related to vasculitis. Initiation of steroid therapy may provide relief of angina in patients with evidence of reversible ischemia in normal coronary arteries.

Aortic coarctation repair--lost and found: the role of local long term specialised care.

BACKGROUND: Aortic coarctation is associated with significant abnormalities of the underlying vasculature. Surgical repair, although relieving obstruction, is not a cure; patients continue to have a high risk of complications with a significantly reduced life expectancy. It has become increasingly apparent that they require regular specialised follow up lifelong. However provision of such services is limited particularly for patients living some distance from the largest cities where specialised adult congenital heart disease services tend to based. We carried out a notes-based study to look at the adequacy of long term follow up in such patients and to assess the role of a local specialist cardiology service in managing these patients. METHODS: We carried out a notes-based study of 55 patients with aortic coarctation referred to a new specialist grown up congenital heart disease clinic based in a large district general hospital over 100 miles from the nearest surgical centre specialising in adult congenital heart disease. RESULTS: A significant proportion of the patients in this study had already suffered major complications by the time of referral. Despite this, nearly half had, at some stage, been lost to follow up and a third had been referred from the community with new complications. 52% of the women had produced children often with little cardiological support. Few patients had had any specialised imaging. At initial review in the clinic, 41% had significant hypertension, although only a small proportion were on antihypertensives. Following initial review in the specialist GUCH clinic, new medications were initiated in 55%, mostly for hypertension; aortic imaging was performed in 94%; and 22% were referred for further specialist investigation or invasive treatment. CONCLUSIONS: This study demonstrates that many patients with previous repair of aortic coarctation have not received optimal long term care. Many had been lost to regular cardiology follow up and, even amongst those who had been seen in cardiology clinics, there was a high frequency of poorly treated or unsuspected complications. The provision of local expert care from a cardiologist specialising in congenital heart disease allows earlier and more aggressive treatment of complications and may also improve compliance. Where a localised specialist is not available, general cardiologists need to work to protocol-driven care pathways with easy access to specialist support.