poole society aug 28 2016

CT Vascular Lesions on Computed Tomography Angiography

Dr. Jayanth H. Keshavamurthy M.D.Assistant professor of Radiology.Medical College of Georgia.Augusta University.

No disclosures

Case 1

• A 39-year-old woman presented with a history of chest pain, palpitations, and syncope. Cardiac gated CTA was performed.

Cardiac gated CTAPersistence of levo atrial cardinal vein with an interatrial course and normal coronary arteries.

• The levo atrial cardinal vein is a very rare embryonic remnant.

• It originates from a pulmonary vein or the left atrium and drains into the superior vena cava or the innominate vein.

• It usually persists with an obstruction to the outflow of the left atrium, in cases of mitral stenosis or atresia, and aortic stenosis or atresia.

• It has also been reported to be associated with cor triatriatum

• Although persistence of the levoatrial cardinal vein is associated with a left to right shunt, our patient was hemodynamically stable and remained asymptomatic and no further evaluation was necessary.

• In cases of a significant left to right shunt through a persistent levoatrial cardinal vein, the treatment options are either surgical ligation or percutaneous placement of an Amplatz occluder device.

• It is important to recognize this embryologic abnormality and to report it in patients who need an open-heart surgery, to alert the surgeon to be aware of this anomalous venous structure.

http://www.journalofcardiovascularct.com/article/S1934-5925(16)30146-0/fulltext

Case 2

• A 44 year old African American woman presents to the hospital with right flank pain, fever, fatigue and nausea.

• Review of systems was positive for dark urine, rash over arms and legs, discoloration of ears with burning and shoulder and knee arthralgia's.

Photograph

• Ears

CTA abdomen

Angiogram

?

Levamisole induced vasculitis.

• Urine drug screen was positive for cocaine and urinary detection of levamisole by gas chromatography confirming cocaine adulteration.

J Clin Rheumatol. 2016 Jan;22(1):47-8. doi: 10.1097/RHU.0000000000000342.Another Vasculitis Red Herring: Spontaneous Renal Artery Bleed Associated With Levamisole-Adulterated Cocaine.Machua W1, Oliver AM.

Case 3

• Abdominal CTA for bleeding and pain abdomen

MIP

Parkes Weber syndrome

• Parkes Weber syndrome is characterized by vascular abnormalities known as capillary malformations and arteriovenous fistulas (AVFs), which are present from birth.

History and etymology

• Frederick Parkes Weber (1863-1962), physician and dermatologist; London England. In 1918, Weber noted the association of this triad with arteriovenous fistulas.

• In 1900, the French physicians Klippel and Trénaunay first described a syndrome characterized by a capillary nevus of the affected extremity, lateral limb hypertrophy, and varicose veins.  

• The capillary malformations increase blood flow near the surface of the skin.

• They usually look like large, flat, pink stains on the skin, and because of their color are sometimes called "port-wine stains."

• Parkes Weber syndrome, capillary malformations occur together with multiple micro-AVFs, which are tiny abnormal connections between arteries and veins that affect blood circulation.

• These AVFs can be associated with life-threatening complications including abnormal bleeding and heart failure.

• Another characteristic feature of Parkes Weber syndrome is overgrowth of one limb, most commonly a leg.

• Abnormal growth occurs in bones and soft tissues, making one of the limbs longer and larger around than the corresponding one.

• Most cases of Parkes Weber syndrome occur in people with no history of the condition in their family. These cases are described as sporadic.

• Some cases of Parkes Weber syndrome result from mutations in the RASA1 gene. When the condition is caused by RASA1 gene mutations, affected individuals usually have multiple capillary malformations.

Case 4

• 60 y/o man with a history of chronic calcific pancreatitis with sinistral portal hypertension due to splenic vein thrombosis.

Splenic venous thrombosis development of gastric and/or gastroepiploic varices

• Greenwald and Wasch first outlined the pathophysiology of left-sided portal hypertension in 1939.

• The name sinistral portal hypertension is a misnomer since portal pressure is usually within the normal range in these cases.

• Other synonymous terminologies referring to sinistral portal hypertension are left sided portal hypertension, segmental, regional, localized, compartmental, lineal, or splenoportal hypertension.

Chronic calcific pancreatitis Tortuous varices in the splenic bed

area and gastroepiploic venous region and no esophageal varices

Absence of cirrhosis, ascites, and portal hypertension.

Splenomegaly Umbilical vein recanalization was

not visualized.

Symptoms

• It is a rare cause of upper gastric bleeding.

Sinistral Portal Hypertension causes:

• Pancreatic diseases: – Acute or Chronic Pancreatitis – Pancreatic pseudocyst– Pancreatic cancer (more common cause)

• Adenopathy from metastatic carcinoma or lymphoma • Iatrogenic causes :

– Splenectomy– Partial gastrectomy– Distal splenorenal shunt

• Blood from the spleen flows towards the stomach via the short gastric veins and then flows towards the portal vein.

• Collateral circulation from the short gastric vein leads to the dilation of stomach submucosal vessels and subsequently increased blood flow and venous pressure, resulting in isolated gastric varices.

• These isolated gastric varices are the characteristic clinical feature of SPH, and may cause life-threatening gastric bleeding.

• The incidence of gastric bleeding in SPH varies from 4 to 72 % .

Treatment• It is important to differentiate between sinistral and

generalized/mesenteric portal hypertension since the optimal management of each disease process is quite different. 

• Treatment options for refractory haemorrhage from varices in cirrhotic patients, specifically endoscopic clipping or TIPS,

will have NO benefit in sinistral hypertension.

• Splenic arterial embolization and partial splenic embolization have each been reported effective for the treatment of acute gastric bleeding due to SPH

Treatment

• Mostly conservative management.• Rarely splenectomy.• Prophylactic splenectomy in patients without upper GI

bleeding is not recommended.

• Wang Q, Xiong B, Zheng C, Liang M, Han P. Splenic Arterial Embolization in the Treatment of Severe Portal Hypertension Due to Pancreatic Diseases: The Primary Experience in 14 Patients. Cardiovascular and Interventional Radiology. 2016;39:353-358. doi:10.1007/s00270-015-1199-8.

Case 5

• PET CT performed for weight loss.

• Incidental double aortic arch and esophageal narrowing. No malignancy identified.

• CTA , Upper GI and cardiac catheterization performed.• Surgically corrected successfully.

Dysphagia lusoria

• Dysphagia lusoria, first described in 1794, describes compression of the esophagus by anomalies of aortic root and arch vasculature.

• Most common anomaly resulting in symptomatic dysphagia is aberrant origin and retroesophageal course of the right subclavian artery.

• Double aortic arch (DAA) occurs less than 0.3% of population but often found in infancy.

• Dysphagia lusoria secondary to DAA is rare, especially as a late presentation.

• Pathophysiology of late onset dysphagia remains unclear. Hypertension and atherosclerosis leads to dilation of aortic arch causing mechanical compression has been proposed as a possible explanation.

• Diagnostic evaluation often includes barium swallow to demonstrate the stenosis and confirmed by CT/MR angiography which clarifies the anatomy of aortic arch.

• Symptomatic cases often require surgical intervention. • Repair of DAA involves division of non-dominant arch.

Kanne JP, Godwin JD. Right aortic arch and its variants. J Cardiovasc Comput Tomogr. 2010 Sep-Oct;4(5):293-300

Case 6

• 6 months after aortic valve replacement a cardiac CTA was obtained for atrial fibrillation for pulmonary vein mapping

Post operative appearance of a periaortic seroma

Discussion

• Most commonly seen after aortic grafts and endovascular graft replacements.

• Here patient was managed conservatively.• Differential diagnosis is infection and if suspected

nuclear medicine scanning may help confirm this finding

Case 7

• Abdominal CTA with 3 D volume rendering and intraluminal views for endovascular coiling planning of a renal artery aneurysm.

Fig. 1a Coronal computed tomography MIP view of a left renal artery aneurysm. The patient has three right renal arteries. b Three-dimensional reconstruction CT view of the left renal artery aneurysm. c MRP reformat with virtual intraluminal view at the same level (d) demonstrate the aorta (Ao) and the left renal artery (arrow in c and “RA” in d). eMRP reformat of the aneurysm with a virtual intraluminal view at the same level (f) demonstrate the left renal aneurysm (An) and the left renal artery distal to the aneurysm (arrow in e and “RA” in f)

http://dx.doi.org/10.1007/s10554-016-0930-0

Case 8

• Young patient with history of hypertension, and neurofibromatosis I

MIP

T2 Pre op MRI

Diagnosis?

Mid aortic syndrome.

Post op VR image after left nephrectomy and a patent bypass graft.

Midaortic syndrome

• Midaortic syndrome is rare, found in < 10% of NF patients, and is commonly associated with renal artery stenosis as in this patient.

• It typically leads to hypertension that manifests in childhood.

• On histopathology, one can find intimal proliferation and the presence of neurofibromas.

• The loss of neurofibromin gene encoded by NF1 gene that is regularly expressed by endothelial cells to regulate cell proliferation may lead to unregulated smooth muscle proliferation.

• NF1 presents itself in a variety of different manifestations, with the most common being café au lait spots, neurofibromas, and Lisch nodules.

• Surgery is superior in providing a longer time before reintervention

• Patient has since undergone aortic valve replacement for a bicuspid aortic valve.

• Name the association between abdominal coarctation and a bicuspid aortic valve?

Shone’s Complex• Shone's syndrome is an uncommon congenital anomaly described

by Shone first in 1963. • The incidence of Shone's syndrome commonly known as Shone's

complex or Shone's anomaly is 0.6% of all congenital heart diseases.

• The characteristic four lesions are coarctation of aorta, subaortic stenosis, parachute mitral valve (PMV), and supramitral ring.

• In a complete Shone's complex all the four pathological lesions are present, while in an incomplete case three or less pathological lesions are present. 

Case 9

• What is the incidental finding on this CT chest

Persistent left SVC and right SVC draining to left atrium

• Almost 40% of patients with PLSVC can have a variety of associated cardiac anomalies, such as atrial septal defect, bicuspid aortic valve, coarctation of aorta, coronary sinus ostial atresia, and cor triatriatum.

• PLSVC has various practical implications when the left subclavian vein is used for access to the right side of the heart or pulmonary vasculature.

• During cardiac surgery, the presence of PLSVC is a relative contraindication to the administration of retrograde cardioplegia.

Case 10

• What is the congenital heart disease ?

Chest Radiographs 5 years apartDescribe the shape of the heart and the pulmonary vascularity.

Uncorrected tetralogy of Fallot

• Infundibular stenosis• RVH• VSD• Overriding of aorta• Major aorto pulmonary collateral arteries

• Now not treatable, too late, mother refused surgery.

• Tetralogy of Fallot (TOF) is the most common congenital cyanotic heart defect, occurring in 3/10,000 live births and accounting for 7-10% of all congenital heart defects.

• If there is ASD also- it is called pentalogy of Fallot.

Answer- Boot shaped heart ( coeur en sabot) and decreased pulmonary vascularity.

• The toe of the boot is formed by the upward pointing cardiac apex, which makes an acute angle with the diaphragm.

• The upturned cardiac apex is ascribed to right ventricular hypertrophy and occurs in 65% of patients with TOF .

• The narrower upper part of the boot results from a small or absent main pulmonary artery partly caused by a narrow infundibulum and hypo plastic main pulmonary artery.

• The latter has been confirmed at angiography in 57% of patients .

Case 11

• Due to chronic cough, upper airway noise, and dysphagia, a 17-month old boy went to pediatrics otolaryngology and underwent laryngoscopy and bronchoscopy that confirmed airway obstruction with mid-tracheal flattening.

• CTA chest performed.

Diagnosis?

Vascular rings

• The two most common forms of complete vascular rings,

• double aortic arch and • right aortic arch with retroesophageal left

subclavian and left ligamentum arteriosum, • make up almost 95% of the cases.

• Thus, complete vascular rings comprised of right aortic arch with mirror-image branching and left ligamentum arteriosum are said to be extremely rare.

• Here, there is no retro esophageal left subclavian artery.

• To summarize, the complete vascular ring consists of: the aortic arch on the right connected to the ligamentum arteriosum running posterior to the esophagus wrapping around the left side to attach to the pulmonary trunk which, along with the aorta, attach to the heart anteriorly completing the ring.

• The axial CT image and 3D volume rendered CT images clearly show the right sided arch with mirror image branching pattern consisting of a left innominate artery coming off the arch first that branches into the left common carotid and left subclavian arteries followed by the right common carotid and then finally the right subclavian coming off the arch last.

• Additionally, the CT images demonstrated the presence of a ligamentum arteriosum coursing behind the esophagus from a Kommerell diverticulum to the pulmonary trunk.

Right aortic arch

• Right Aortic Arch

• Types– At least five different types– Only two of importance

• Mirror Image Type — Type I• Aberrant left subclavian — Type II

• Associated with cardiac defects 5-10% of the time– Tetralogy of Fallot most often (71%)– ASD or VSD next most often  (21%)– Coarctation of aorta rarely (7%)

Right arch with mirror image branching.

• One of the rarest causes of a complete vascular ring.• Fortunately, this anatomical anomaly was recognized

early on, and at 2 years of age the patient successfully underwent surgical resection of the diverticulum and ligamentum which provided complete decompression of the esophagus and trachea and relief of all prior respiratory and upper gastrointestinal symptoms.

Kanne JP, Godwin JD. Right aortic arch and its variants. J Cardiovasc Comput Tomogr. 2010 Sep-Oct;4(5):293-300

Case 12

Crossing vessel sign

Case 13

Shortness of breath

• PH is defined as a mean pulmonary artery (PA) pressure —assessed by right cardiac catheterization — ≥ 25 mmHg at rest.

• Idiopathic PH is a rare condition, with an incidence of 2.5 to 3.5 cases per million population in Western countries.

• It is 2 or 3 times more common in women than in men, at any age, and peaks in the 4th, 5th and 6th decades of life. PH associated with other conditions is more common, although its actual incidence in those cases is unknown.

Vascular changes• Initially, the first radiological sign assessed was the PA diameter.

Main PA diameter should be measured in its bifurcation, at a right angle to its long axis and just lateral to the ascending aorta.

• According to Kuriyama 22, a PA diameter ≥ 29 mm has a PPV of 0.97, 87 % sensitivity and 89 % specificity in predicting PH; for this reason, this value has been used as indicative of PH .

• However, it has been proven that absolute measures are not completely reliable, as PA pressure and size depend on the body mass, sex and age. It seems to be increased by 6 % in healthy individuals over the age of 50 and in obese patients.

• It also increases with exercising and in athletes.

• Specificity climbs to 100 % when a PA diameter ≥ 29 mm is accompanied by a segmental artery-to-bronchus ratio > 1:1 in most lung lobes.

• It is better to compare the main PA with the adjacent aorta (Ao) and if the PA/Ao ratio is > 1, PH is very likely, particularly in patients below the age of 50.

Egg and banana sign

• Another recent parameter is the assessment of the PA at the level of the aortic arch (egg —PA— and banana —Ao— signs) , seen in severe PH.

• Within the parenchymal vessels, Sheehan reports the phenomenon of “neovascularization”, which describes serpiginous and fine peripheral vessels that frequently emerge from the centrilobular arterioles, without following the normal anatomy of the pulmonary vessels.

• Originally, Sheehan described these findings in idiopathic PH and in the Eisenmenger syndrome; however, they seem to be a manifestation of all-cause severe PH .

• Peripheral anastomoses of systemic intercostal vessels that supply the lung periphery.

Cardiac changes

• Structural changes of the right chambers include hypertrophy and RV dilatation, right atrium(RA) enlargement and functional tricuspid regurgitation, secondary to valvular ring dilatation.

• Progressive increase in RV pressure damages the structure and function of the left ventricle (LV), which reduces its size and distorts

• Increased RV wall thickness. Values > 4 mm are considered pathological .

• RA enlargement and regurgitation of contrast agent into the IVC and hepatic veins is indicative of tricuspid valve insufficiency with 90 % sensitivity and 100 %.

• Vena cava and azygos vein dilatation is considered a marker of right-sided hypertension.

• In addition to this, Isaacs proposes the assessment of coronary sinus distension that is easily identifiable on almost all CT studies. Dilatation > 11mm is associated with increased PA pressures, confirmed by catheterization.

• The RV/LV size ratio needs to be measured. This may be done on axial slices, but four-chamber plane measurements correlate better with echocardiographic measurements. Under normal conditions, LV diameter is wider than that of RV.

• Interventricular septum position: As the pressure in the RV increases, the normal position of the septum varies and at very high pressures septal inversion occurs, resulting in LV dysfunction, with reduction of its volume/minute (output), which is a very serious condition. Septal inversion is a bad prognostic sign.

• • Pericardial thickening or effusion. It is more common and earlier visualized in the anterior or aortopulmonary recess, where it appears in the shape of a “bikini bottom”. It may be considered an indirect sign of PH, particularly if associated with increased PA diameter.

• It is not necessarily associated with pleural effusion. It is also a sign of RV dysfunction and of bad prognosis.

Case 14

• A patient with a history of a repaired congenital heart disease and status post a dual-chamber internal cardioverter defibrillator (ICD) placement presented with chest pain.

• A chest radiograph revealed, that the right atrial (RA) lead was coursing posteriorly instead of the normal anterior course.

• Computed tomography (CT) confirmed that the RA bipolar screw-in pace/sense lead was positioned in a left juxtaposed right atrial appendage (JRAA).

Fig. 1 Frontal (A) and lateral (B) radiographs demonstrate the distal tip of the bipolar screw-in pace/sense lead located in the left JRAA (long arrow). The conduit from the RV to the main PA (curved arrow) and the abandoned epicardial leads (short arrow) are shown. Axial (C) and coronal (D) CT images demonstrate the distal tip of the bipolar screw-in pace/sense lead in the left JRAA (arrow). The curved arrow in the axial CT demonstrates the occluded RV to PA conduit.

Left juxtaposed right atrial appendage (JRAA).

• Juxtaposition of atrial appendages are often associated with conotruncal abnormalities, double-outlet right ventricle, transposition of the great arteries, dextrocardia, tricuspid atresia or severe stenosis, hypoplastic right ventricle, and bilateral conus .

• JRAA is associated with a high prevalence of other cardiac malformations that are usually cyanotic and alter the atrial septal plane.

• Knowledge of the latter is of great importance when performing cardiac surgery, invasive procedures like an atrial balloon septostomy, or implantation of a cardiac pacemaker or defibrillator.

Case 15

• A 38-year-old male with a history of multiple recurrent biatrial myxomas, presents after 4 prior resections. His physician requests an evaluation of a Left Atrial Myxoma and the vascular supply to the myxoma in anticipation of a 5th resection.

EKG: Supraventricular Tachycardia, a markedly posterior QRS axis, and ST depression

Blue naevi of the trunkLentigines of the face

Gross Image of Myxoma from Biopsy

Histology of Myxoma from Biopsy (200X Magnification)

Histology of Myxoma from Biopsy (500X Magnification)

Atrial Myxoma of the Left Atrium visualized in the axial and coronal plane, measuring 41.0 mm x 23.5 mm

• Atrial Myxomas occur most frequently in patients with no family history of cardiac myxomas, with 76% occurring in women with a mean age of 51 years, and 86% occurring in the left atrium.

• Due to the unusual nature of recurrent myxomas, it is suspected that the patient suffers from Carney’s Complex, an autosomal dominant inherited syndrome normally characterized by recurrent myxomas or schwannomas, endocrine neoplasms, and skin nevi and lentigines on the face and trunk.

Carney triad (CT)

• Characterized by the coexistence of three types of neoplasms, mainly in young women, including

• gastric gastrointestinal stromal tumor,• pulmonary chondroma, and • extra-adrenal paraganglioma.

Carney complex

• Myxomatous neoplasms (cardiac, endocrine, cutaneous and neural), and a host of pigmented lesions of the skin and mucosae, including the rarely occurring epitheloid blue nevus.

Myxoma syndrome• LAMB (lentigines, atrial myxomas, mucocutaneous

myxomas, and blue naevi)• NAME (naevi, atrial myxomas, myxoid neurofibroma,

and ephelides (freckles))• Carney syndrome (atrial, cutaneous and mammary

myxomas, lentigines, blue naevi, endocrine disorders and testicular tumours)

• Identification of a Novel Genetic Locus for Familial Cardiac Myxomas and Carney Complex

• Mairead Casey, Caroline Mah, Andrew D. Merliss, Lawrence S. Kirschner,Susan E. Taymans, Alfred E. Denio, Bruce Korf, Alan D. Irvine, Anne Hughes, J. Aidan Carney, Constantine A. Stratakis and Craig T. Basson.

• Circulation. 1998;98:2560-2566, published online before print December 8, 1998

•  http://dx.doi.org/10.1161/01.CIR.98.23.2560