Multiple Pulmonary Arteriovenous Malformations: An Unusual Cause of Shortness of Breath and Recurrent Strokes.

OBJECTIVE: To discuss an uncommon case of a patient with multiple pulmonary arteriovenous malformations (PAVMs) presenting with dyspnea on exertion and recurrent strokes. BACKGROUND: A 79-year-old woman with recent onset recurrent cerebrovascular accidents (CVAs) was referred to cardiology for evaluation of dyspnea on exertion. Clinical examination was unrevealing. METHODS: A transthoracic echocardiogram (TTE) with agitated saline was suggestive of an extra-cardiac shunt. Subsequent chest computed tomographic angiography (CTA) demonstrated five large PAVMs. RESULTS: The patient underwent transcatheter coil embolization of the five PAVMs, resulting in marked improvement in dyspnea and resolution of the shunt on bubble study echocardiography. CONCLUSIONS: Although PAVMs are rare, they remain an important entity to consider when evaluating patients with extracardiac shunts. They may present with nonspecific features such as dyspnea on exertion and recurrent CVAs. All patients with multiple PAVMs should undergo thorough skin screening for telangiectasia as well as radiologic imaging to screen for additional AVMs in the liver and brain in order to exclude hereditary hemorrhagic telangiectasia (HHT).

Sinus Venosus Atrial Septal Defect Complicated by Eisenmenger Syndrome and the Role of Vasodilator Therapy.

Sinus venosus atrial septal defect is a rare congenital, interatrial communication defect at the junction of the right atrium and the vena cava. It accounts for 5-10% of cases of all atrial septal defects. Due to the rare prevalence and anatomical complexity, diagnosing sinus venous atrial septal defects poses clinical challenges which may delay diagnosis and treatment. Advanced cardiac imaging studies are useful tools to diagnose this clinical entity and to delineate the anatomy and any associated communications. Surgical correction of the anomaly is the primary treatment. We discuss a 43-year-old Hispanic female patient who presented with dyspnea and hypoxia following a laparoscopic myomectomy. She had been diagnosed with peripartum cardiomyopathy nine years ago at another hospital. Transesophageal echocardiography and computed tomographic angiography of the chest confirmed a diagnosis of sinus venosus atrial septal defect. She was also found to have pulmonary arterial hypertension and Eisenmenger syndrome. During a hemodynamic study, she responded to vasodilator and she was treated with Ambrisentan and Tadalafil. After six months, her symptoms improved and her pulmonary arterial hypertension decreased. We also observed progressive reversal of the right-to-left shunt. This case illustrates the potential benefit of vasodilator therapy in reversing Eisenmenger physiology, which may lead to surgical repair of the atrial septal defect as the primary treatment.

Ascending thoracic aorta: postoperative imaging evaluation.

Advances in computed tomography (CT) scanners and electrocardiographic gating techniques have resulted in superior image quality of the ascending aorta and increased the use of CT angiography for evaluating the postoperative ascending aorta. Several abnormalities of the ascending aorta and aortic arch often require surgery, and various open techniques may be used to reconstruct the aorta, such as the Wheat procedure, in which both an ascending aortic graft and an aortic valve prosthesis are implanted; the Cabrol and modified Bentall procedures, in which a composite synthetic ascending aorta and aortic valve graft are placed; the Ross procedure, in which the aortic valve and aortic root are replaced with the patient's native pulmonary valve and proximal pulmonary artery; valve-sparing procedures such as the T. David-V technique, which leaves the native aortic valve intact; and more extensive arch repair procedures such as the elephant trunk and arch-first techniques, in which interposition or inclusion grafts are implanted, with or without replacement of the aortic valve. Normal postoperative imaging findings, such as hyperattenuating felt pledgets, prosthetic conduits, and reanastomosis sites, may mimic pathologic processes. Postoperative complications seen at CT angiography that require further intervention include pseudoaneurysms, anastomotic stenoses, dissections, and aneurysms. Radiologists must be familiar with these procedures and their imaging features to identify normal postoperative appearances and complications.

Imaging findings in human Bordetella bronchiseptica pneumonia.

Bordetella bronchiseptica is a gram-negative coccobacillus, which causes respiratory infection in dogs, rabbits, and pigs, and is rarely a human pulmonary pathogen. We present 2 immunocompromised patients diagnosed with B. bronchiseptica pneumonia. To our knowledge, these are the first reports of this disease in patients with B-cell lymphoma and an orthotopic cardiac transplant. In this report, we briefly discuss the epidemiology, microbiology, and challenges in the laboratory identification of B. bronchiseptica and describe the protean imaging manifestations of this rare pulmonary infection. We also address issues related to treatment and prognosis.

Fibropolycystic liver disease in children.

Fibropolycystic liver diseases are a group of associated congenital disorders that present most often in childhood. These disorders include congenital hepatic fibrosis, biliary hamartomas, autosomal dominant polycystic liver disease, choledochal cysts and Caroli disease. We present a discussion and illustrations of the embryology, genetics, anatomy, pathology, imaging approach and key imaging features that distinguish fibropolycystic liver disease in children. The pathogenesis of these disorders is believed to be abnormal development of the embryonic ductal plates, which ultimately form the liver and biliary systems. An understanding of the abnormal embryogenesis helps to explain the characteristic imaging features of these disorders.