CVS Flashcards
CVS
A 30-year-old man has been involved in an Road Traffic Accident (RTA). Aortic injury is suspected. CT angiogram shows a fusiform dilatation at the anteromedial aspect of the aortic isthmus with a steep contour superiorly, gently merging with the proximal descending thoracic aorta inferiorly. What is the likely diagnosis?
A. Pseudoaneurysm
B. Coarctation of the aorta
C. Ductus diverticulum
D. Aortic nipple
E. Avulsed left subclavian artery
C. Ductus diverticulum
Ductus diverticulum is a focal bulge at the anteromedial aspect of the aortic isthmus, visualized in 9% of adults. It is critical to identify this normal variant and distinguish it from a post-traumatic false aneurysm, which also occurs most commonly at the aortic isthmus (88%). The classic ductus diverticulum has smooth, uninterrupted margins and gently sloping symmetric shoulders; in contrast, false aneurysms have a variety of shapes and sizes with sharp margins and often contain linear defects. Compared with the classic ductus diverticulum, the atypical ductus diverticulum has a shorter and steeper slope superiorly and a more classic gentle slope inferiorly. However, both shoulders have smooth, uninterrupted margins, an important feature that distinguishes this variant from true injury. Other normal variants that can mimic injury include aortic spindle, which is a smooth circumferential bulge immediately distal to the aortic isthmus; infundibulum at the origin of aortic branches like the brachiocephalic and intercostal arteries, which are spherical or conical in shape but have a vessel at its apex, thereby differentiating them from false aneurysms.
CVS
An obese 25-year-old man presents with atypical chest pain. Cardiac MR demonstrates asymmetrical hypertrophy of the interventricular septum, primarily affecting the anteroinferior portion. What is the most likely diagnosis?
A. Hypertrophic obstructive cardiomyopathy
B. Restrictive cardiomyopathy
C. Myocardial infarction
D. Dilated cardiomyopathy
E. Constrictive pericarditis
A. Hypertrophic obstructive cardiomyopathy
Hypertrophic cardiomyopathy (HCM) is defined as a diffuse or segmental left-ventricular hypertrophy with a non-dilated and hyperdynamic chamber, in the absence of another cardiac or systemic disease explaining the degree of cardiac muscle hypertrophy. Dyspnoea on exertion is the most common symptom because the key functional hallmark of hypertrophic cardiomyopathy is an impaired diastolic function with impaired LV filling in the presence of preserved systolic function. Systolic dysfunction occurs at end-stage disease. Asymmetric involvement of the interventricular septum is the most common form of the disease, accounting for an estimated 60%–70% of the cases of HCM.
Other variants include apical, symmetric, midventricular, mass-like and non-contiguous HCM is typically associated with hypertrophy of the muscle to 15 mm or thicker and a ratio of thickened myocardium to normal left-ventricular basal myocardium of 1.3–1.5. With MRI and multidetector computed tomography (CT), apical HCM has a characteristic spade like configuration of the LV cavity at end diastole, appreciated on vertical long-axis views.
CVS
A 58-year-old woman undergoes an echocardiogram followed by cardiac MRI for investigation of exertional dyspnoea. The cardiac MRI was reviewed at the X-ray meeting, and the radiologist diagnosed concentric hypertrophic cardiomyopathy. Which of the following did the radiologist see?
A. Thickening of the interatrial septum at 7 mm
B. Thickening of the entire LV wall measuring 17 mm at end diastole
C. Nodular high signal in the interventricular septum on T2
D. Thickening of the LV wall measuring 14 mm with normal systolic function
E. Thickened LV with delayed hyperenhancement of mid wall
B. Thickening of the entire LV wall measuring 17 mm at end diastole
HCM should be differentiated from other causes of symmetric increased thickness of the LV wall, including athlete’s heart, amyloidosis, sarcoidosis, Fabry disease and adaptive LV hypertrophy due to hypertension or aortic stenosis. HCM is associated with hypertrophy of the muscle to 15 mm or thicker. In cardiac amyloidosis, the amyloid protein is deposited in the myocardium, which leads to diastolic dysfunction and restrictive cardiomyopathy. Because amyloidosis is a systemic process, involvement of all four chambers is common; thus, an increase in the thickness of the interatrial septum and right atrial free wall by more than 6 mm is seen. Dynamic enhanced MRI shows late enhancement over the entire subendocardial circumference. Sarcoidosis is a non-caseating granulomatous disease that infiltrates any area of the body, but most of the morbidity/mortality is from involvement of the heart. MRI shows nodular or patchy increased signal intensity on both T2-weighted and enhanced images, which often involves the septum (more particularly, the basal portion) and the LV wall, whereas papillary and right-ventricular infiltration are rarely seen.
Fabry disease is a rare X-linked autosomal recessive metabolic storage disorder. At MRI, the LV wall is seen to be concentrically thickened, and delayed hyperenhancement is typically seen mid-wall and has been reported in the basal inferolateral segment. Differentiation between compensatory hypertrophy and HCM is sometimes difficult. In comparison to HCM, patients with compensatory hypertrophy usually have normal systolic function, rather than hyperdynamic systolic function in HCM, and their LV wall rarely exceeds 15 mm in maximal thickness. Athlete’s heart can show increased LV wall thickness but end diastolic volume and ejection fraction are normal. Another feature of the cardiac remodeling in athletes is the lack of areas of delayed hyperenhancement within the LV myocardium at dynamic enhanced MRI.
CVS
A 30-year-old woman presents with shortness of breath and fatigue. CT shows enlargement of the right atrium, right ventricle and pulmonary artery and normal appearance of the left atrium. What is the most likely diagnosis?
A. VSD – Ventricular Septal Defect
B. ASD – Atrial Septal Defect
C. Bicuspid aortic valve
D. Coarctation of the aorta
E. Mitral valve disease
B. ASD – Atrial Septal Defect
LA LV RA RV * VSD x x x * Uncomplicated ASD x x * ASD with shunt reversal (Eisenmenger syndrome) x x x * Mitral valve disease x x * Tricuspid valve disease x x * Pulmonary hypertension x x * PDA x
CVS
A 3 year-old presents as acutely unwell with a maculopapular rash, lymphadenopathy and erythema of her palms. Her white cell count is normal, and a specific cause for her symptoms is not found. She improves on immunoglobulins and supportive treatment. A follow-up echocardiogram shows cardiomegaly and a coronary artery aneurysm. What is the
likely diagnosis?
A. Takayasu arteritis
B. Kawasaki arteritis
C. Moyamoya syndrome
D. Henoch-Schönlein purpura
E. Churg-Strauss syndrome
B. Kawasaki arteritis
Kawasaki disease is a systemic vasculitis that is more severe in small and medium arteries, and veins to a lesser extent, with inflammatory’ lesions in virtually all organs. It is a leading cause of acquired heart disease in childhood. The aetiology of KD remains unknown, although the clinical presentation - self-limiting illness manifested by an abrupt onset of fever, rash, exanthema, conjunctival injection and cervical adenopathy - and the epidemiological features - a seasonal peak in winter and spring, age distribution and a geographic wave-like spread of illness during epidemics - strongly suggest an infectious cause.
Fever is usually the first sign of KD. Rash is non-specific and mostly maculopapular. Cervical lymphadenopathy is the last common of the main manifestations. Cardiovascular complications include coronary artery aneurysms, myocarditis, pericarditis with pericardial effusion, systemic arterial aneurysms, valvular disease, mild aortic root dilatation and myocardial infarct.
Takayasu arteritis (TA), also known as pulseless disease, is a granulomatous large vessel vasculitis that predominantly affects the aorta and its major branches, with increased prevalence in Asian women <50 years of age.
Churg Strauss syndrome is a small-to-medium vessel necrotizing pulmonary vasculitis, affecting patients in the third and fourth decades with asthma, eosinophilia and systemic symptoms like purpura and arthralgia.
Moyamoya disease is an idiopathic, non-inflammatory, non-atherosclerotic, progressive vasculo- occlusive disease involving the circle of Willis, typically the supra-clinoid internal carotid arteries.
It has a bimodal age distribution, affecting children and adults. In children, ischaemic strokes are most pronounced, whereas in adults haemorrhage from the abnormal vessels is more common.
CVS
A 76-year-old male patient with chronic inflammatory disease and known history of secondary generalized multisystem amyloidosis showed an abnormal appearance of the heart on echocardiography. Dynamic enhanced cardiac MR imaging was advised for further characterization. All of the following are imaging findings seen with cardiac amyloidosis, except
A. Left ventricular wall hypertrophy
B. Subendocardial delayed myocardial hyperenhancement
C. Systolic dysfunction
D. Granular echogenic myocardium
E. Interatrial septal thickening
C. Systolic dysfunction
In cardiac amyloidosis, the amyloid protein is deposited in the myocardium, which leads to diastolic dysfunction that progresses to restrictive cardiomyopathy. Because amyloidosis is a systemic process, involvement of all four chambers is common; thus, an increase in the thickness of the interatrial septum and right atrial free wall by more than 6 mm has been shown to be a specific finding for cardiac amyloidosis. Through the use of dynamic enhanced cardiac MRI, a distinct pattern of late enhancement, which was distributed over the entire subendocardial circumference, has been shown to have high specificity and sensitivity for cardiac amyloidosis
Echocardiogram shows concentric LV hypertrophy, with hyperechoic granular sparkling of the ventricular wall.
CVS
A child with exertional dyspnoea and abnormal chest X-ray showing a boot-shaped heart and oligaemic lungs is diagnosed as suffering from tetralogy of Fallot. The pulmonary oligaemia is secondary to right ventricular outflow tract (RVOT) obstruction. Which of the following is the most common implicated cause for obstruction of RVOT?
A. Hypoplastic pulmonary annulus
B. Pulmonary valvular stenosis
C. Infundibular stenosis
D. Combined infundibular and pulmonary valvular stenosis
E. Overriding ventricular septum
C. Infundibular stenosis
Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease. This disease accounts for approximately 10% of all congenital heart defects, affecting men and women equally. In addition, TOF is the most common cyanotic heart disease that survives to adulthood.
The four components of TOF, first described in 1888 by French physician Etienne-Louis Arthur Fallot, are interventricular communication (ventricular septal defect), right-ventricular outflow tract (RVOT) obstruction, concentric right-ventricular hypertrophy (RVH) and deviation of the origin of the aorta to the right.
Combined infundibular and pulmonary valvular stenosis is the second most common cause.
CVS
A 3-year-old child with shortness of breath is diagnosed with tetralogy of Fallot. All of the following abnormalities may be associated with this condition, except
A. Transposition of great vessels (TGA)
B. Patent ductus arteriosus (PDA)
C. Anomalous origin of coronary arteries
D. DiGeorge syndrome
E. Right-sided aortic arch
A. Transposition of great vessels (TGA)
A number of associated features can occur in patients with Tetralogy of Falot (TOF). Right sided aortic arch is the most common variant, known as Corvisart syndrome. Coronary artery anomalies, such as the left anterior descending artery arising from the right coronary artery (whose course may run directly across the right ventricular outflow tract) can occur. Other associations include patent ductus arteriosus, multiple ventricular septal defects and complete atrioventricular septal defect. Approximately 15% of patients have extracardiac anomalies, including chromosomal abnormalities such as Down’s syndrome, DiGeorge syndrome and Alagille syndrome.
IR
A 66-year-old joiner presents to his GP with jaundice and abdominal discomfort. He was subsequently referred to a gastroenterologist who requests a liver biopsy due to deranged liver function tests. Which of the following options is not a contraindication for percutaneous liver biopsy?
A. INR above 1.6
B. Platelets less than 60,000/mm3
C. Tense ascites
D. Extra-hepatic biliary obstruction
E. Suspected haemangioma
E. Suspected haemangioma
Contraindications for liver biopsy include the following:
1. Uncooperative patient
2. Extrahepatic biliary duct dilatation (except if benefit outweighs the risk)
3. Bacterial cholangitis (relative contraindication due to risk of septic shock)
4. Abnormal coagulation indices (having a normal INR or PT is not a reassurance that the patient will not bleed; however, there is increased incidence of bleeding with INR above 1.5)
5. Thrombocytopenia (platelet count below 60,000/mm3)
6. Presence of ascites
7. Cystic lesion
CVS
A 56-year-old woman with an increase in shortness of breath comes to the A&E department and is assessed by the physicians. The ECG is low in voltage and a chest X-ray is organised. The chest X-ray shows a very large heart with sharply defined borders and a narrow pedicle, suggesting pericardial effusion. All of the following are associated, except
A. Tuberculosis pericarditis
B. Blunt trauma to the sternum
C. Hyperthyroidism
D. Radiation pericarditis
E. Pericardial lymphoma
C. Hyperthyroidism
Inflammation of the pericardium (pericarditis) occurs in response to a variety of stimuli. It results in cellular proliferation or the production of fluid (pericardial effusion), either alone or in combination. Causes include myocardial infarction (acute or post-myocardial infarction Dressier syndrome), pericardiotomy, mediastinal irradiation, infection (viral or bacterial), connective-tissue disease (rheumatoid arthritis, SLE), metabolic disorders (uraemia, hypothyroidism rather than hyperthyroidism), pericardial neoplasia, trauma and AIDS.
Chest X-ray shows increased cardiac size, ‘flask’ or ‘water bottle’ configuration, filling of retrosternal space, effacement of cardiac borders and thickening of anterior pericardial stripe. Echocardiography is the investigation of choice for diagnosis.
CVS
A 66 year-old man with progressive shortness of breath and low-volume ECG shows
an enlarged heart on chest X-ray. Echocardiogram confirms the presence of a moderately large pericardial effusion. The pericardial fluid is aspirated for symptomatic relief and sent off for cytology and culture. Cytology comes back as positive for malignant cells. Which of the following is the most common type of primary pericardial malignancy?
A. Fibrosarcoma
B. Pericardial angiosarcoma
C. Fibromyxoid sarcoma
D. Mesothelioma
E. Epithelioid endothelioma
D. Mesothelioma
Malignant mesothelioma is the most common primary pericardial malignancy. A causal relationship with asbestosis is uncertain because of low prevalence of this neoplasm. Mesothelioma may present as a well-defined single mass, multiple nodules, or diffuse plaques involving the visceral and parietal pericardium and wrapping around the cardiac chambers and great vessels.
Other malignant primary tumours include lymphoma, sarcoma, pheochromocytoma and liposarcoma. Teratomas of the pericardium may also be malignant and arc most commonly seen in children.
Pericardial metastases are much more common than primary pericardial tumours. Breast and lung cancers are the most common sources of metastases in the pericardium, followed by lymphomas and melanomas.
CVS
A 35-year-old woman undergoes an X-ray in the A&E department with suspicion of chest infection. The X-ray reveals an abnormal mediastinal opacity but no evidence of chest infection. Review of two old films done 2 and 6 years ago shows the same abnormal mediastinal opacities with no significant interval change in size, shape, appearance or location. Judging by its location, the radiologist reports it as a simple pericardial cyst or spring water cyst. Which of the following statements concerning congenital simple pericardial cysts is false?
A. They are homogenous and well defined on frontal chest X-ray.
B. They are most commonly left-sided.
C. On MRI, they are low’ on T1W and high on T2W images.
D. Pericardial cysts can contain proteinaceous material.
E. Pericardial cysts can occasionally calcify.
B. They are most commonly left-sided.
The most common congenital pericardial anomaly is a pericardial cyst. Chest pain is the most common presenting symptom, but most patients with pericardial cysts are asymptomatic. On plain chest radiographs, pericardial cysts present as well-defined, round, homogeneous soft-tissue densities and are most commonly found at the right pericardiophrenic angle. Pericardial cysts are visualised most easily using CT or MRI. With MRI, simple pericardial cysts are characterised by low signal intensity on T1-weighted images or high signal intensity on T2 -weighted images. With CT, pericardial cysts are usually of water density, but when they contain sufficient proteinaceous material the attenuation may be greater than that of water.
Occasionally, pericardial cysts may calcify and simulate thymic cysts.
CVS
A 34-year-old woman with chest pain, shortness of breath and collapse is brought to the A&E department. Initial chest X-ray is abnormal. Subject to the abnormal appearance of the cardiac contour, an MRI is obtained in the local cardiac centre on the following day, which confirms a large congenital pericardial defect. All of the following are imaging features, except:
A. Abnormal cardiac contour on plain chest X-ray.
B. Failure to identify pericardium on CT or MR is diagnostic.
C. Most commonly, a left-sided location.
D. Shift of cardiac axis to the left.
E. Association with ASD.
B. Failure to identify pericardium on CT or MR is diagnostic.
Congenital pericardial defects are uncommon. They range from small defects to complete absence of the pericardium. Both small pericardial defects and complete absence of the pericardium most often are left-sided. They can be recognised on plain chest radiographs because there is abnormal cardiac contour due to protrusion of all or part of the cardiac chamber, for example, the left atrial appendage, through the defect. Shift of the cardiac axis to the left and posteriorly is seen with complete absence or large pericardial defects. With CT or MRI, failure to visualise a portion of the pericardium does not necessarily indicate a pericardial defect, however, because the pericardium over the left atrium and ventricle may not always be visualised in normal subjects.
Patients with pericardial defects also may have one or more associated congenital abnormalities, including atrial septal defect, patent ductus arteriosus, mitral valve stenosis, or TOF, which also are detectable on CT or MRI.
CVS
A 66-year-old woman with progressive shortness of breath, reduced exercise tolerance and occasional chest pain shows engorged neck veins and hepatomegaly on clinical examination. She is clinically thought to have constrictive pericarditis. All of the following are imaging features of constrictive pericarditis, except
A. Pericardial thickness of more than 4 mm.
B. Pericardial thickening may be limited to the right side of the heart.
C. MR is better at demonstrating pericardial calcification.
D. Sigmoid-shaped ventricular septum.
E. Increased diameter of the IVC.
C. MR is better at demonstrating pericardial calcification.
Patients with constrictive pericarditis present with symptoms of heart failure. The most frequent causes are cardiac surgery and radiation therapy. Other causes include infection (viral or tuberculous), connective-tissue disease, uraemia, neoplasm, or idiopathic condition.
Transthoracic echocardiography is not very accurate in the depiction of pericardial thickening. Transoesophageal imaging allows better visualisation of the pericardium, and Doppler techniques are particularly useful in the diagnosis; however, the transoesophageal approach has a narrow field of view and is invasive.
Both CT and MRI demonstrate the pericardium very well. Normal pericardial thickness is less than 2 mm. Pericardial thickness of 4 mm or more indicates thickening and, when accompanied by features of heart failure, is suggestive of constrictive pericarditis. Constrictive pericarditis and restrictive cardiomyopathy are differentiated on the basis of thickened pericardium.
Pericardial thickening may be limited to the right side of the heart or an even smaller area, such as the right atrioventricular groove. An additional advantage of CT is its high sensitivity in depicting pericardial calcification. It is important to remember, however, that neither pericardial thickening nor calcification is diagnostic of constrictive pericarditis unless the patient also has symptoms of physiologic constriction or restriction.
At both CT and MRI, the right ventricle tends to have a reduced volume and a narrow tubular configuration. In some patients, a sigmoid-shaped ventricular septum or prominent leftward convexity in the septum can be observed. Systemic venous dilatation particularly inferior vena cava (IVC), hepatomegaly and ascites also are frequently seen.
CVS
A young woman presents to the AED following a scuffle on a night out. On examination, there is a suspected fifth metacarpal fracture of her right hand. A plain radiograph is subsequently organized. This does not demonstrate a fracture, but it is noted that the patient has relatively short fourth metacarpal bones. Old chest films show bilateral inferior rib notching involving the third to sixth ribs bilaterally. What is the likely diagnosis?
A. Noonan syndrome
B. Turner syndrome
C. Pseudohypoparathyroidism
D. Marfan syndrome
E. Achondroplasia
B. Turner syndrome
Turner syndrome is a female, sex chromosome abnormality from the deletion of one X chromosome (45 XO). It is characterised by a webbed neck and short stature. Skeletal manifestations include short fourth metacarpals and Madelung’s deformity. 5-20% of patients with Turner syndrome have coarctation of the aorta, which would account for the additional finding of rib notching. Pseudohypoparathyroidism and pseudopseudohypoparathyroidism can exhibit a short fourth metacarpal, but they are not associated with coarctation of the aorta. Noonans syndrome is associated with short stature and characteristic facies, along with other congenital cardiopulmonary anomalies, but shortening of the fourth metacarpal is not a feature of this entity. Marfan syndrome is associated with pectus excavatum and aortic root dilatation with the increased risk of aortic dissection.
