Takayasu Arteritis: JACC Focus Seminar 3/4.

Takayasu arteritis is a rare idiopathic large-vessel vasculitis that typically affects young women. An early "prepulseless" stage is often missed, associated with nonspecific constitutional symptoms (fever, malaise, and weight loss) and elevated inflammatory markers. Unchecked disease progression leads to the "pulseless" stage, manifest clinically by missing pulses, vascular tenderness, and ischemic symptoms (limb claudication, dizziness, angina, and renovascular hypertension), and is characterized pathologically by arterial wall thickening and stenotic/occlusive lesions or aneurysm formation. Vascular complications (stroke, blindness, heart failure, and aneurysm rupture) could follow unless disease progression is halted by immunosuppressive therapy and critical lesions are palliated by timely endovascular therapy or open surgery. Early diagnosis, effective therapy, and lifelong surveillance for disease activity relapses and vascular disease progression are critical to successful long-term outcomes. The outlook for patients has improved significantly in recent years with the establishment of diagnostic and classification criteria, better investigational modalities, and more effective medical and invasive therapy.

Coronary artery disease management and cost implications with fractional flow reserve guided coronary intervention in Indian patients with stable ischemic coronary artery disease.

OBJECTIVES: To study the safety of stent avoidance, frequency of change in management decisions, and its cost implications while using a fractional flow reserve (FFR)-guided treatment strategy for intermediate-grade coronary artery stenosis. BACKGROUND: The impact of FFR in guiding management decisions and its cost implications has not been studied after imposition of a ceiling on stent prices by the Government of India. METHODS: In 400 patients with 477 intermediate-grade coronary lesions for whom coronary intervention was planned, functional assessment using FFR was done. Incidence of the primary composite endpoint (major adverse cardiac event [MACE], cardiac death, myocardial infarction, objective evidence of ischemia, and target vessel revascularization) in the stent avoided subset was compared with the stented group at follow-up. Micro-costing analysis was done using a computed model with current stent and FFR wire prices. RESULTS: The overall incidence of MACE was 4.9%, 0.9% in the stent-avoided subset and 6.9% in stented group (p = 0.04, comparing the latter two) at a median follow-up of 21 months (interquartile range 12-31 months). Serious adverse events occurred only in 1% of patients receiving adenosine. The average cost saving was Indian rupees (INR) 51,847 [United States Dollar (USD) 746] per patient, resulting in total savings of INR 15,813,379 (USD 227,530). Cost savings persisted but were lower by 36% (INR 18,613/USD 268 per patient) after the ceiling of stent prices. CONCLUSION: FFR-guided percutaneous coronary intervention (PCI) strategy is safe and cost-effective in countries where majority of patients self-finance their health care, resulting in stent and PCI avoidance in approximately one in three patients referred for coronary angioplasty.

Type 3 Coronary Perforation on Optical Coherence Imaging.

OCT imaging revealed a 1.5 mm-long transmural breach in a segment of the artery devoid of calcium or eccentric plaque burden. Focal negative remodeling identified on OCT was deemed the reason for this otherwise unexplained cause of coronary rupture.

Cardiac involvement in beta-thalassaemia: current treatment strategies.

Despite the advances in the management of thalassemia major, heart disease remains the leading cause of mortality in patients afflicted with this disorder. Cardiac involvement in thalassemia encompasses a spectrum of disorders including myocardial dysfunction, arrhythmias, pulmonary hypertension, and peripheral vascular disease. Although cardiac siderosis (accumulation of iron in cardiac myocytes) as a consequence of repeated blood transfusions is deemed to be the main etiologic factor for myocardial dysfunction in transfusion-dependent patients, the significance of other pathophysiologic mechanisms is being increasingly recognized especially in non-transfusion dependent patients. Management of cardiac complications in thalassemia major hinges on the treatment of the underlying pathophysiology, which often is unmitigated iron overload. The prevalence and predictors of cardiac complications in 'ex-thalassaemics' [thalassaemic patients undergoing allogeneic hematopoietic stem cell transplantation (HSCT) is unknown at present. In this review, we look at the pathogenesis of cardiac involvement in patients with beta-thalassemia major, the advances in the management of these patients and the future prospects.

Malignant multivessel coronary spasm complicated by myocardial infarction, transient complete heart block, ventricular fibrillation, cardiogenic shock and ischemic stroke.

Multivessel coronary spasm resulting to cardiogenic shock and malignant ventricular arrhythmias though rare has been reported in the literature. The disease seems to be more prevalent in Asians. There have been isolated reports of coronary spasm in patients with reactive airway disease. We report the first case of spontaneous multivessel spasm in a male patient with bronchial asthma of Arab ethnicity resulting in acute myocardial infarction complicated by cardiogenic shock, recurrent ventricular arrhythmias, and transient complete heart block. Literature review of similar cases suggests a strong association with bronchial asthma and a more malignant course in patients with reactive airway disease. The role of intracoronary nitroglycerin in proving the diagnosis even in patients in shock on maximal inotropic supports and intra-aortic balloon pump is highlighted and the importance of considering multivessel coronary spasm as a cause for acute coronary syndrome even in patients with conventional risk factors for atherosclerotic coronary artery disease is reinforced in the discussion of this case.

Aortic annulus dimension assessment by computed tomography for transcatheter aortic valve implantation: differences between systole and diastole.

Accurate assessment of aortic annular dimensions is essential for successful transcatheter aortic valve implantation (TAVI). Annular dimensions are conventionally measured in mid-systole by multidetector computed tomography (MDCT), echocardiography and angiography. Significant differences in systolic and diastolic aortic annular dimensions have been demonstrated in cohorts without aortic stenosis (AS), but it is unknown whether similar dynamic variation in annular dimensions exists in patients with severe calcific AS in whom aortic compliance is likely to be substantially reduced. We investigated the variation in aortic annular dimensions between systole and diastole in patients with severe calcific AS. Patients with severe calcific AS referred for TAVI were evaluated by 128-slice MDCT. Aortic annular diameter was measured during diastole and systole in the modified coronal, modified sagittal, and basal ring planes (maximal, minimal and mean diameters). Differences between systole and diastole were analysed by paired t test. Fifty-nine patients were included in the analysis. Three of the five aortic dimensions measured increased significantly during systole. The largest change was a 0.75 mm (3.4%) mean increase in the minimal diameter of the basal ring during systole (p = 0.004). This corresponds closely to the modified sagittal view, which also increased by mean 0.42 mm (1.9%) during systole (p = 0.008). There was no significant change in the maximal diameter of the basal ring or the modified coronal view during systole (p > 0.05). There is a small magnitude but statistically significant difference in aortic annulus dimensions of patients with severe AS referred for TAVI when measured in diastole and systole. This small difference is unlikely to alter clinical decisions regarding prosthesis size or suitability for TAVI.

Percutaneous pericardio-peritoneal shunt (PPP)--a novel palliative treatment for recurrent cardiac tamponade.

The management of pericardial effusion with recurrent cardiac tamponade in disseminated malignancy is a therapeutic challenge. Treatment options include repeated aspirations, balloon pericardiostomy and surgical pericardial window with variable success at symptom palliation. We describe a safe and novel percutaneous technique of pericardio-peritoneal shunt for palliative treatment of malignancy associated massive pericardial effusion ina patient who declined surgery and had refractory pericardial effusion despite repeated pericardiocentesis, chemical pericardiodesis and balloon pericardiostom