Percutaneous Intervention of Cabrol Graft-Left Main Anastomosis.

The Cabrol technique involves surgical reimplantation of coronary arteries after aortic root replacement. It uses a Dacron graft interposed between the aortic root graft and the native coronary artery. A stenosis of the graft-coronary anastomosis requires either surgical or percutaneous correction. An understanding of the Cabrol and modified Cabrol techniques and the associated anatomy is essential for a successful percutaneous intervention. We report a case of percutaneous intervention of a Cabrol graft-left main coronary artery stenosis in a patient who presented with exertional angina.

Fontan-Associated Dyslipidemia.

Background Hypocholesterolemia is a marker of liver disease, and patients with a Fontan circulation may have hypocholesterolemia secondary to Fontan-associated liver disease or inflammation. We investigated circulating lipids in adults with a Fontan circulation and assessed the associations with clinical characteristics and adverse events. Methods and Results We enrolled 164 outpatients with a Fontan circulation, aged ≥18 years, in the Boston Adult Congenital Heart Disease Biobank and compared them with 81 healthy controls. The outcome was a combined outcome of nonelective cardiovascular hospitalization or death. Participants with a Fontan (median age, 30.3 [interquartile range, 22.8-34.3 years], 42% women) had lower total cholesterol (149.0±30.1 mg/dL versus 190.8±41.4 mg/dL, P<0.0001), low-density lipoprotein cholesterol (82.5±25.4 mg/dL versus 102.0±34.7 mg/dL, P<0.0001), and high-density lipoprotein cholesterol (42.8±12.2 mg/dL versus 64.1±16.9 mg/dL, P<0.0001) than controls. In those with a Fontan, high-density lipoprotein cholesterol was inversely correlated with body mass index (r=-0.30, P<0.0001), high-sensitivity C-reactive protein (r=-0.27, P=0.0006), and alanine aminotransferase (r=-0.18, P=0.02) but not with other liver disease markers. Lower high-density lipoprotein cholesterol was independently associated with greater hazard for the combined outcome adjusting for age, sex, body mass index, and functional class (hazard ratio [HR] per decrease of 10 mg/dL, 1.37; 95% CI, 1.04-1.81 [P=0.03]). This relationship was attenuated when log high-sensitivity C-reactive protein was added to the model (HR, 1.26; 95% CI, 0.95-1.67 [P=0.10]). Total cholesterol, low-density lipoprotein cholesterol, and triglycerides were not associated with the combined outcome. Conclusions The Fontan circulation is associated with decreased cholesterol levels, and lower high-density lipoprotein cholesterol is associated with adverse outcomes. This association may be driven by inflammation. Further studies are needed to understand the relationship between the severity of Fontan-associated liver disease and lipid metabolism.

A "Cat"-astrophic Case of Bartonella henselae infective endocarditis followed by cardiac transplantation salvage therapy.

To our knowledge, no cases of Bartonella henselae endocarditis leading to subsequent heart transplantation salvage therapy have been published. We present a case of a 29-year-old man with cat-inflicted B henselae endocarditis and concurrent worsening heart failure, who then underwent successful heart transplantation 50 days following diagnosis. Treatment and monitoring strategies used in this patient are discussed. Furthermore, we review literature related to heart transplantation salvage therapy for endocarditis due to other intracellular pathogens.

A novel approach to percutaneous removal of large tricuspid valve vegetations using suction filtration and veno-venous bypass: A single center experience.

BACKGROUND: Tricuspid valve surgery has been the de facto standard treatment for tricuspid valve endocarditis (TVE) refractory to medical therapy. It is now possible to remove right-sided vegetations percutaneously using a venous drainage cannula with an extracorporeal bypass circuit. OBJECTIVES: The purpose of our study is to describe our single-center experience of percutaneous tricuspid valve vegetation removal. METHODS: We reviewed the perioperative course of 33 consecutive patients with large tricuspid valve vegetations who carried high surgical risk. RESULTS: The cohort included 12 males and 21 females over a 40-month period with an average age of 37 years. A preponderance of patients carried an admitted or confirmed diagnosis of injection drug use (72.7%). Average vegetation size was 2.1 +/- 0.7 cm prior to the procedure with a 61% reduction in size after the procedure. All patients survived the procedure and 90.9% survived the index hospitalization. Three patients proceeded to elective tricuspid valve replacement due to worsening severity of tricuspid regurgitation. CONCLUSION: Percutaneous removal of large tricuspid valve vegetations is a safe and effective alternative for patients with TVE who carry high-surgical risk. © 2017 Wiley Periodicals, Inc.

Percutaneous Intervention to Treat Platypnea-Orthodeoxia Syndrome: The Toronto Experience.

OBJECTIVES: This study reviewed a series of patients treated with transcatheter closure of septal defect to treat platypnea-orthodeoxia syndrome, with specific attention to septal characteristics and device choice. BACKGROUND: Platypnea-orthodeoxia syndrome is an uncommon condition characterized by positional dyspnea and hypoxemia due to intracardiac right-to-left shunting through a patent foramen ovale (PFO), an atrial septal defect, or pulmonary arteriovenous malformations. Percutaneous closure of such defects is the treatment of choice. METHODS: In this single-center series, 52 patients were treated with percutaneous closure of an interatrial communication after presentation between January 1997 and July 2015. Septal morphology, clinical, procedural, and outcomes data were analyzed. RESULTS: All patients had a PFO; however, nearly one-quarter required a non-PFO device (11 Amplatzer Septal Occluder and 1 post-infarct muscular VSD), as opposed to a dedicated PFO device to achieve shunt occlusion. These patients were characterized by an aneurysmal septum, shorter primum septum overlap with the secundum septum, and greater septal angulation from the midline. After closure, all demonstrated acute improvements in oxygen saturation (pre-procedure: 81 ± 8%; post-procedure: 95.1 ± 0.5% on room air). Each patient was treated with a single device and no one required re-intervention. CONCLUSIONS: Patients presenting with platypnea-orthodeoxia syndrome can be treated successfully with a percutaneous intervention often requiring a variety of devices. Those requiring a non-PFO-type device had a greater prevalence of an aneurysmal septum, shorter primum septal overlap with the secundum septum, and greater septal angulation with the midline.

Worsening Hypoxia Post Lung Transplant: What has Changed?

Platypnoea-orthodeoxia is a rare, but under-diagnosed clinical entity, characterised by postural hypoxia and breathlessness. Underlying pathology is inter-atrial shunt or pulmonary vascular malformation, but what anatomical distortion / physiological alterations initiates right to left shunt, usually against the pressure gradient remains unknown.

Mediastinal Fibrosis of the Pulmonary Artery Secondary to Tuberculosis.

Mediastinal fibrosis is an uncommon disease involving the esophagus, respiratory tract, and great vessels. We report a man who presented with dyspnea on exertion. Computed tomography of the chest demonstrated granulomatous disease with dense calcifications leading to severe stenosis of the main pulmonary artery (PA) and narrowing of the superior vena cava. The results of tuberculosis (TB) interferon-γ release assay and TB-polymerase chain reaction were positive for Mycobacterium tuberculosis. The patient received 2 weeks of treatment for latent TB before undergoing resection of fibrotic tissue and replacement of the main and branch PAs using a homograft.

Acid sphingomyelinase promotes lipoprotein retention within early atheromata and accelerates lesion progression.

OBJECTIVE: The key initial step in atherogenesis is the subendothelial retention of apolipoprotein B-containing lipoproteins. Acid sphingomyelinase (acid SMase), an enzyme present extracellularly within the arterial wall, strongly enhances lipoprotein retention in model systems in vitro, and retained lipoproteins in human plaques are enriched in ceramide, a product of SMase. We now sought to test a direct causative role for acid SMase in lipoprotein retention and atherogenesis in vivo. METHODS AND RESULTS: We studied atherogenesis and lipoprotein retention in Asm(-/-) versus Asm(+/+) mice on the Apoe(-/-) and Ldlr(-/-) backgrounds. Asm(-/-);Apoe(-/-) mice had a approximately 40% to 50% decrease in early foam cell aortic root lesion area compared with Asm(+/+);Apoe(-/-) mice (P<0.05) despite no difference in plasma cholesterol or lipoproteins. To assay lipoprotein retention in vivo, the two groups of mice were injected with fluorescently labeled Apoe(-/-) lipoproteins. Early foam cell lesions of Asm(-/-);Apoe(-/-) mice showed a striking 87% reduction in lipoprotein trapping (P<0.0001) compared with Asm(+/+);Apoe(-/-) lesions. Similar results were obtained with Ldlr(-/-) mice, including an 81% reduction in lipoprotein retention within Asm(-/-);Ldlr(-/-) lesions compared with Asm(+/+);Ldlr(-/-) lesions (P<0.0005). CONCLUSIONS: These findings support a causal role for acid SMase in lipoprotein retention and lesion progression and provides further support for the response-to-retention model of atherogenesis.

Suppression of macrophage eicosanoid synthesis by atherogenic lipoproteins is profoundly affected by cholesterol-fatty acyl esterification and the Niemann-Pick C pathway of lipid trafficking.

Atheroma macrophages internalize large quantities of lipoprotein-derived lipids. While most emphasis has been placed on cholesterol, lipoprotein-derived fatty acids may also play important roles in lesional macrophage biology. Little is known, however, about the trafficking or metabolism of these fatty acids. In this study, we first show that the cholesterol-fatty acyl esterification reaction, catalyzed by acyl-CoA:cholesterol acyltransferase (ACAT), competes for the incorporation of lipoprotein-derived fatty acids into cellular phospholipids. Furthermore, conditions that inhibit trafficking of cholesterol from late endosomes/lysosomes to the endoplasmic reticulum (ER), such as the amphipathic amine U18666A and the Npc1+/- mutation, also inhibit incorporation of lipoprotein-derived fatty acids into phospholipids. The biological relevance of these findings was investigated by studying the suppression of agonist-induced prostaglandin E(2) (PGE(2)) and leukotriene C(4)/D(4)/E(4) production during lipoprotein uptake by macrophages, which has been postulated to involve enrichment of cellular phospholipids with non-arachidonic fatty acids (NAAFAs). We found that eicosanoid suppression was markedly enhanced when ACAT was inhibited and prevented when late endosomal/lysosomal lipid trafficking was blocked. Moreover, PGE(2) suppression depended entirely on acetyl-LDL-derived NAAFAs, not on acetyl-LDL-cholesterol, and was not due to decreased cPLA(2) activity per se. These data support the following model: lipoprotein-derived NAAFAs traffic via the NPC1 pathway from late endosomes/lysosomes to a critical pool of phospholipids. In competing reactions, these NAAFAs can be either esterified to cholesterol or incorporated into phospholipids, resulting in suppression of eicosanoid biosynthesis. In view of recent evidence suggesting dysfunctional cholesterol esterification in late lesional macrophages, these data predict that such cells would have highly suppressed eicosanoid synthesis, thus affecting eicosanoid-mediated cell signaling in advanced atherosclerosis.