ABSTRACT
Interrupted aortic arch (IAA) is a rare congenital heart condition where there is a complete discontinuation between the ascending and descending aorta. The association with a patent ductus arteriosus or developed arterial collateral allows survival until adulthood in extremely rare cases. We report a case series of adult forms of IAA. Even if the incidence is very rare, IAA should be could excluded in the setting of resistant asymmetrical hypertension. The first case is singular regarding its association with a bicuspid aortic valve and aortic aneurysm, while the second case is characterized by massive left ventricular hypertrophy. The diagnosis was suspected on echocardiographic findings and confirmed by computed tomography angiography. Both patients refused surgical repair given the high operative risk. Learning objectives: -The evaluation of severe resistant high blood pressure should include complementary investigation for adult form interrupted aortic arch.-To understand the major impact of multimodality imaging for the recognition, localization, and stratification of interrupted aortic arch.
ABSTRACT
BACKGROUND: Fractional flow reserve (FFR) guided-percutaneous interventions is nowadays the gold standard for optimal coronary artery revascularization. While multiple pressure guidewires have been validated, the use of microcatheter for FFR measurements is still a matter of debate. OBJECTIVE: The aim of this study was to investigate the crossing profile characteristics of the NAVVUS® microcatheter as compared with the COMETTM pressure wire. At the same time, we compared non-hyperaemic pressure ratio (NHPR) and FFR measurements of both systems. METHOD/MATERIALS: In this retrospective study, all angiographically intermediate coronary artery stenoses each month were assessed, using either the NAVVUS® or the COMETTM pressure system, to receive coronary artery physiology assessment with NHPR and FFR measurements. The crossing profile of both systems was compared regarding objective coronary artery lesion characteristics using quantitative coronary analysis evaluation. RESULTS: Over a period of 4.5 years, we evaluated 213 coronary artery stenoses using one of the two coronary tools. We found a 9.2% crossing profile failure rate using the microcatheter, compared to 0.7% in the pressure wire group (p < .001). The crossing failure was significantly correlated with the presence of coronary artery calcifications and angulation (p = .042, p = .049, respectively). FFR values were comparable be- tween the two groups and were significantly lower in the presence of coronary calcifications and proportional to the degree of stenosis (p = .036, p = .010). Pressure drift was comparable. CONCLUSION: Our observations are in line with other studies reporting the poor crossing profile of the NAVVUS® microcatheter. NHPR and FFR measures of both systems were well correlated.
ABSTRACT
Hemodynamical evaluation of a coronary artery lesion is an important diagnostic step to assess its functional impact. Fractional flow reserve (FFR) received a class IA recommendation from the European Society of Cardiology for the assessment of angiographically moderate stenosis. FFR evaluation of coronary artery disease offers improvement of the therapeutic strategy, deferring unnecessary procedures for lesions with a FFR > 0.8, improving patients' management and clinical outcome. Post intervention, an optimal FFR > 0.9 post stenting should be reached and > 0.8 post drug eluting balloons. Non-hyperemic pressure ratio measurements have been validated in previous studies with a common threshold of 0.89. They might overestimate the hemodynamic significance of some lesions but remain useful whenever hyperemic agents are contraindicated. FFR remains the gold standard reference for invasive assessment of ischemia. We illustrate this review with two cases introducing the possibility to estimate also non-invasively FFR from reconstructed 3-D angiograms by quantitative flow ratio. We conclude introducing a hybrid approach to intermediate lesions (DFR 0.85-0.95) potentially maximizing clinical decision from all measurements.
ABSTRACT
BACKGROUND: Systemic juvenile lupus erythematosus can affect any organ including the heart. While pericarditis and endocarditis are the most common cardiac complications, dilated cardiomyopathy remains rare. We report the full recovery of dilated cardiomyopathy in a girl with juvenile lupus. CASE PRESENTATION: A 7-year-old girl presented with persistent fever, weight loss, alopecia, positive anti-lupus antibodies, and decreased complement. Examination found dyspnea, tachycardia, and hypotension. Chest X-ray revealed an enlarged cardiac silhouette, and echocardiography showed a left ventricular ejection fraction of 35%. After excluding other causes, systemic lupus erythematosus related dilated cardiomyopathy was selected. Heart failure treatment, Prednisone, and hydroxychloroquine were prescribed. On day three, she developed macrophage activation syndrome which responded to methylprednisolone boluses. Clinical and biological improvement was observed after 4 weeks, normalization of echocardiography after 4 months. CONCLUSION: A review of the literature confirmed the rare and severe nature of dilated cardiomyopathy in juvenile lupus emphasizing the importance of performing echocardiography at the slightest cardiac sign.
