Management of Marfan Syndrome during pregnancy: A real world experience from a Joint Cardiac Obstetric Service.

BACKGROUND: Pregnancy in Marfan Syndrome (MFS) is associated with increased maternal risk of cardiovascular events. Given the maternal and genetic risks, pre-conception counselling is essential to facilitate informed choices. Multidisciplinary antenatal care with regular imaging is mandatory and best delivered through a Joint Cardiac Obstetric Service (JCOS). The aim of this study was to compare the care delivered in a JCOS against recognised international standards (European Society of Cardiology (ESC)). METHODS: Pregnancies in women with MFS from 2005 to 2015 were identified from our institutional database. Patient records were reviewed and practice assessed against pre-determined standards based on ESC guidelines. RESULTS: There were 23 pregnancies in 15 women with MFS. 13/23 (57%) occurred in women with aortic dilatation at baseline. There were 3 important maternal cardiac events (type A dissection; deterioration in left ventricular function; significant left ventricular and progressive aortic dilatation). Four women did not have access to expert pre-conception counselling. These women were all referred to the JCOS late in established pregnancy. Imaging was often delayed and only 7/23 cases (30%) met the standard for minimum frequency of echocardiographic surveillance. Only 12/23 (52%) had pre-conception imaging of the whole aorta with CT/MRI. Distal aortic dilatation was identified in 7/23 cases but none of these underwent further MRI evaluation during pregnancy. CONCLUSION: Despite having a dedicated JCOS, our data show that facilitating complete obstetric and cardiac care for this group remains challenging. Education of local care providers and timely referral for expert pre-conception counselling in a JCOS are key.

New technologies aimed at percutaneous intervention in the small coronary artery.

Percutaneous coronary intervention (PCI) of small vessels can be complicated by technically difficult access to the target lesion, an increased risk of major adverse cardiac events and in-stent restenosis requiring repeat revascularization. Conventional management of such lesions is with drug-eluting stent implantation; however, these have only partly attenuated the problem. In response, several medical device companies are competing to produce new technologies aimed at PCI in small coronary arteries. Such innovations include thin-strutted stents, stent-on-a-wire systems, drug-coated balloons, endothelial progenitor cell-catching stents and biodegradable stent systems. To date, none of these techniques have been sufficiently validated for use in small coronary arteries to justify a change in practice; however, small-vessel coronary artery disease is an increasingly common problem, and PCI of target lesions with reference vessel diameter <3.0 mm is likely to increase, especially in view of the increasing prevalence of diabetes, warranting further well-designed studies. The prospect of mounting a self-expandable biodegradable drug-eluting stent directly onto a guidewire could potentially be an exciting future development.

Anomalous origination of a coronary artery from the opposite sinus.

Anomalous origination of a coronary artery from the opposite sinus (ACAOS) is estimated to be present in 0.2-2.0% of the population. In the majority of individuals, ACAOS has no hemodynamic or prognostic implications, but in a minority of cases, typically where the anomalous coronary artery takes an interarterial course to reach its correct myocardial territory, it can precipitate ischemia and sudden cardiac death (SCD). With the growing use of CT coronary angiography (CTCA) in the investigation of ischemic heart disease, we can expect increasing rates of incidental detection of this anomaly. Although CTCA and magnetic resonance coronary angiography can effectively characterize these lesions anatomically, they fail to describe and quantitatively assess the basic defect that leads to coronary insufficiency, such as mural intussusception. The key challenge lies in the identification of which patients are at risk of SCD and, therefore, who should be offered corrective surgical or (potentially) percutaneous intervention. Conventional, noninvasive stress testing has limited sensitivity, but emerging, invasive stress tests, which utilize intravascular ultrasonography and measurements of fractional flow reserve, show the potential to provide more-accurate hemodynamic and prognostic assessment.