Image of the Month April 2018 - Absence doesn't make the heart grow fonder..

Absence doesn’t make the heart grow fonder:  an interesting case of partial pericardium.

Dr Tom Chance1, Dr Laura Duerden1, Dr Jonathan Rodrigues2, Dr Stephen Lyen1, Dr M Hamilton1, Dr N Manghat1.

Affiliations:

1 Department of Radiology, Bristol Royal Infirmary, United Kingdom

2 Department of Cardiothoracic Imaging, Toronto General Hospital, Toronto

 

Image focus:

A 45 year old man presented with atrial fibrillationA chest radiograph showed a slightly lobulated contour of the superior left heart border (see figure 1). Transthoracic echocardiography (performed at an outside institution) showed possible right ventricular (RV) mass.  Cardiovascular magnetic resonance was performed to clarify the echocardiographic appearances. It demonstrated abrupt pericardial discontinuity on axial HASTE sequence, with the right-sided pericardial edge causing focal, extrinsic RV compression (Fig 2A). Steady state free precession cine images demonstrated an impaired RV ejection fraction of 34%.  The RV indexed end diastolic volume was 77ml/m2 (normal age-matched range 67-111ml/m2). RV volumes quantified on short axis slices. Left ventricular (LV) morphology and function were normal (LV ejection fraction 45%). Cardiac CT was performed to provide higher spatial resolution detail of the pericardial abnormality. This was performed with retrospective gating with 20% mAs outside of diastolic reconstruction windows. This confirmed a large defect in the anterior and lateral pericardium with complete LV and partial RV herniation (Fig.2B and D). There were no discernible superior pericardial recesses (Fig. 2C) and also evidence of lung invaginating between left inferior pulmonary vein ostium and left atrium (Fig.2B, straight white line), indirectly inferring deficient pericardium in these regions.  There was differential contrast opacification in the RV apex, constrained by the pericardial herniation compared to the remaining RV, implying delayed RV apical emptying but no RV apical thrombus (Fig 2B, *). No other congenital abnormality was demonstrated.   The patient declined surgical pericardioplasty, accepting the risk of potential cardiac and/or vascular strangulation. The atrial fibrillation was medically managed.

Discussion:

Congenital pericardial absence is a rare condition, most frequently affecting the left-sided pericardium with either complete or partial absence and resultant LV herniation. Right-sided pericardial involvement is even less common. We present a rare case of partial absence of both left and right sides of the pericardium with complete LV herniation and partial RV herniation diagnosed non-invasively with multi-modality imaging.

Congenital pericardial absence is believed to result from pleuro-pericardial hypoxia and agenesis due to premature atrophy of the left common cardiac vein and is frequently associated with other congenital anomalies. The unusual pattern of pericardial absence and lack of concomitant congenital defect raises the possibility that the abnormality was acquired in early development, but after completion of cardiac organogenesis in this patient.

 

 

Figure 1 - PA chest radiograph. The heart appears enlarged with evidence of indentation in the superior leftward cardiac contour.

 

 

 

 

Figure 2 A. Axial HASTE image showing pericardium exerting extrinsic mass effect on the RV and apical RV herniation. B. Axial ECG-triggered cardiac CT image confirming the partial pericardial absence and demonstrating retained contrast in the herniated RV apex (*). There is lung invaginating between the left inferior pulmonary vein and left atrium (solid white arrow). C. 2-chamber reformat from ECG-gated cardiac CT showing deficient superior pericardial recesses and the inferior extent of the pericardium (solid white arrow). D. Volume-rendered ECG-triggered CT reconstruction demonstrating the point of RV partial herniation (*).

 

Multiple choice questions

Question  1) Congenital pericardial absence most often occurs in which geographic pattern

A-    Complete absence of the pericardium

B-     Absence of the left pericardium

C-     Absence of the right pericardium.

Answer:  B- Absence of the left pericardium. Most common defect is absence of the left pericardium (up to 70% of cases), followed by right pericardium (17%), and then total absence of the pericardium (9%).

Question 2) Of the above geographic patterns of pericardial absence, which has the best prognosis?

A-    Complete absence of the pericardium

B-     Absence of the left pericardium

C-     Absence of the right pericardium

Answer: A- Complete absence of the pericardium. Herniation of the heart through focal pericardial defects can lead to several complications including tricuspid regurgitation, myocardial strangulation, ischaemia and sudden death. Complete absence of pericardium does not normally require intervention unless complications occur[i].

Question 3) Congenital pericardial defects result from the premature atrophy of which structure?

A-    Left common cardinal vein

B-     Right common cardinal vein

C-     Left primitive jugular vein

D-    Left vitelline vein

Answer: A- Left common cardinal vein (duct of Cuvier). As a result, reduced blood supply to the left pleuro-pericardial membrane results in pericardial agenesis[ii].

Question 4 ) Which echocardiographic finding is not typically seen in congenital partial pericardium?

A-    Cardioptosis

B-     Abnormal interventricular septal motion

C-     Apparent right ventricular dilatation

D-    Pulmonary regurgitation

Answer: D- Pulmonary regurgitation. Echocardiographic findings are related to increased cardiac mobility within the chest cavity[iii].

References


[i] Congenital absence of the pericardium. Hyun-Jin Kim, Young-Seok Cho et al. J Cardiovasc Ultrasound. 2014 Mar;22(1):36-39.

[ii] Congenital absence of the pericardium presenting as acute myocardial necrosis. Brulotte S, Roy L et al. Can J Cardiol. 2007 Sep; 23(11): 909-912.

[iii] Congenital absence of left pericardium. Victor AR, Osorio P et al. Rev Port Cardiol. 2003 Jun;22(6):801-10.

Image of the Month - March 2018 - Progressive dyspnoea in a patient with a bioprosthetic AVR

Progressive dyspnoea in a patient with a bioprosthetic AVR

Jack PM Andrews, Marc R Dweck, Alastair J Moss.

Centre for Cardiovascular Science, University of Edinburgh, Chancellor’s Building, Royal Infirmary of Edinburgh, 51 Little France Crescent, Edinburgh EH16 4TJ, UK. 

Corresponding Author: Dr. Jack PM Andrews. E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

Introduction

An 85-year old woman presented to the cardiology service with exertional dyspnoea whilst walking on the flat. She underwent a bioprosthetic surgical aortic valve replacement (stentless 25 mm Kohler Elan, Vascuetec) 12 years ago for calcific aortic stenosis. She was otherwise systemically well and had not undergone any recent dental procedures. On examination, she had a collapsing pulse (BP 120/30 mmHg) and grade 3 early diastolic murmur in the aortic area. Electrocardiogram (ECG) demonstrated sinus rhythm with voltage criteria for left ventricular hypertrophy and routine bloods returned a normocytic anaemia with a mildly elevated serum bilirubin (27 umol/L). A transoesophageal echocardiogram (TOE) was performed. (Figure 1)

2018 March IOM 1

Figure 1. Transoeophageal echocardiogram mid-oesophageal AV long axis view (A). 3D echocardiogram short axis view (B).

  

2018 March IOM 2

Figure 2. Cardiac magnetic resonance demonstrated transprosthesis laminar regurgitation jet (A). 3D  cardiac computed tomography coronal reconstruction of the aortic bioprosthesis confirmed the left cusp prolapse [inset] that is independent from a septated LVOT aneurysm (B). Pre TAVR deployment aortogram showing substantial volume of contrast within the left ventricle confirming significant aortic incompetence (C). Post TAVR deployment aortogram; note the lack of contrast within the left ventricle indicating the return of valvular competence and successful TAVR deployment (D).

Clinical Question

What is the aetiology of the bioprosthetic aortic valve dysfunction?

  1. Infective endocarditis
  2. Late suture dehiscence
  3. Leaflet prolapse
  4. Thrombus formation
  5. LVOT aneurysm formation

  Answer – C

 The correct answer is leaflet prolapse. TOE demonstrated a severe central regurgitant jet at the level of the prosthesis, confirmed by the large width of the colour flow jet (>65% of LVOT diameter) and colour M-mode holodiastolic flow reversal across the prosthesis (Figure 1A & B). Pathological regurgitation of prosthetic heart valves can either be central or paravalvular. Paravalvular regurgitation is often related to position of the sewing ring and anchoring tissue in the outflow tract with latent failure of the suture line due to age-related deterioration or LVOT aneurysm formation. Central regurgitation is a common feature of structural valve dysfunction. Distortions in leaflet anatomy due to perforations or thickening of the cusps ultimately result in leaflets prolapsing below the annular plane.

Whilst echocardiography remains the initial first line investigation, adjuvant imaging using cardiac magnetic resonance is increasingly utilised to confirm the severity and location of regurgitant jets (Figure 2A). [1] Bioprosthetic valve thrombosis and infective endocarditis are important differential diagnoses to exclude using modalities with superior spatial resolution such as cardiac computed tomography (Figure 2B). [2]

Figure 2C and 2D demonstrate aortograms both pre and post deployment of transcathether aortic valve replacement (TAVR) illustrating the immediate cessation of valvular incompetence.  The advent of valve-in-valve interventions offers novel strategies for high-risk patients with failing stentless bioprostheses. [3] Confirming the specific aetiology of bioprosthesis dysfunction with multi-modality imaging is a priority to select the most appropriate therapeutic strategy. This patient underwent transcatheter aortic valve implantation (26mm CoreValve Evolut R, Medtronic) with an immediate improvement in symptoms.

REFERENCES

[1]       Salaun E, Jacquier A, Theron A, Giorgi R, Lambert M, Jaussaud N, Hubert S, Collart F, Bonnet JL, Habib G, Cuisset T, Grisoli D. Value of CMR in quantification of paravalvular aortic regurgitation after TAVI. Eur Heart J – Cardiovascular Imaging. 2016;17:41-50.

[2]       Moss AJ, Dweck MR, Dreisbach JG, Williams MC, Mak SM, Cartlidge T, Nicol ED, Morgan-Hughes GJ. Complementary role of cardiac CT in the assessment of aortic valve replacement dysfunction. Open Heart. 2016;3:e000494. Doi: 10.1136/openhrt-2016-000494.

[3]       Duncan A, Davies S, Di Mario C, Moat N. Valve-in-valve transcatheter aortic valve implantation for failing surgical aortic stentless bioprosthetic valves: A single-center experience. J Thorac Cardiovasc Surg. 2015;150:91-98.

Image of the Month - December 2017 - Multimodality imaging in a case of left atrial myxoma

Multimodality imaging in a case of left atrial myxoma

Williams, MG1; Lewis, K2; MacIver, DH3; Sheffield, E4, Gosling, O3

1Department of Cardiology, Bristol Heart Institute, Bristol, UK

2Department of Radiology, Musgrove Park Hospital, Taunton, UK

3Department of Cardiology, Musgrove Park Hospital, Taunton, UK

4Department of Pathology, North Bristol NHS Trust, Bristol, UK

An asymptomatic 52-year-old gentleman was referred to our district general hospital for routine review due to a family history of aortopathy. He had no past medical history and was normotensive. An initial transthoracic echocardiogram (TTE) revealed normal left ventricular function and normal indexed aortic root dimensions.

He returned for a follow up scan 5 years later and he was found to have asymptomatic moderate LV systolic dysfunction on TTE. Cardiovascular magnetic resonance imaging (CMR) was arranged. This confirmed moderate left ventricular impairment (ejection fraction 43%), no regional wall motion abnormality, no late gadolinium enhancement, a bicuspid aortic valve and a mildly dilated aortic root (Figure 1A-B). There was no evidence of an intra or extra-cardiac mass. The patient was commenced on a beta-blocker and angiotensin receptor blocker.  

Two years later (Figure 1C) a repeat TTE now demonstrated a large left atrial mass. He was admitted and a transoesophageal echocardiogram (Figure 1D) and a cardiac CT (Figure 1E-F) were performed which confirmed the presence of a 22 x 15 mm pedunculated left atrial mass attached to the interatrial septum. There was no evidence of metastases. The CT also confirmed normal coronary arteries.  He was discussed with our local cardiothoracic centre and was transferred urgently for surgical resection (Figure 1G). He has made an excellent recovery and subsequent histological analysis confirmed this was a myxoma.

 2017 dec IOTM myxoma final

 

Figure 1:  4 (A) and 3 chamber (B) cardiac MRI images showing dilated aortic root and no evidence of atrial myxoma in 2015. C Subsequent transthoracic echocardiogram (2017) in the parasternal short axis showing echogenic lesion (white arrow) in the left atrium. D Transoesophageal echocardiogram (TOE) demonstrating the pedunculated lesion attached to the interatrial septum. Contrast-enhanced coronary CT angiogram showing the mass in the axial (E) and oblique (F) views. (G) Photograph of excised lesion prior to histological analysis

 

Multiple choice questions:

1. What is the median age of presentation of a myxoma?

A) 20-30 years

B) 30-40 years

C) 40-50 years
D) 50-60 years

E) 60-70 years

 

Answer C)

Cardiac myxomas are seen most frequently in adult females. At presentation, the median age is 49 years. However, cases have been reported in patients under 20 years and over 90 years of age1.

 

2. Which statement regarding myxoma is correct?

A) Myxoma is the most common type of tumour found in the heart.

B) Treatment is urgent due to high risk of fatal embolism and haemodynamic collapse

C) Local recurrence is common and affects >20% of cases

D) Roughly half of myxomas are solitary

E) The right atrium is the most common location

 

Answer B)

Metastatic tumours are 20-40 times more common than myxomas, which are the most common primary cardiac tumour2. Surgical excision should be considered in all patients as a matter of urgency. Death from embolic complications between diagnosis and treatment has been estimated at between 8-10%3.  Local recurrence is rare affecting 3-4% of patients4. 90% of myxomas are solitary and the left atrium is the most common location representing 86% of myxomas1.

 

3. Which of the following statements regarding the appearance of a myxoma on CT is false?

A) They demonstrate high attenuation  on non-contrast CT

B) 10% are calcified

C) Pedunculated appearance

D) Heterogeneous contrast enhancement

E) May be seen to prolapse through the mitral valve

 

Answer A)

Myxomas usually exhibit low attenuation on non-contrast CT. 10% may be calcified, more commonly when they are located in the right atrium. They are often pedunculated and attached to the interatrial septum via a stalk. They enhance heterogeneously due to the individual tumours’ chronicity and degree of necrosis and haemorrhage. Large tumours may prolapse through the mitral valve and can cause LV inflow obstruction2.

 

References:

1Mir IA et al. Atrial myxoma ‘a review’ International journal of community medicine and public health 2016 Jan; 3(1):23-29

2Kassop D et al. Cardiac Masses on Cardiac CT: A Review. Current Cardiovascular Imaging Reports. 2014;7(8):9281. doi:10.1007/s12410-014-9281-1.

3Dato GMA et al.  Long term follow up of cardiac myxoma, (7-31years). Journal of Cardiovascular Surgery. 1993;34(2):114-43.

4Castells E et al. Cardiac myxoma: Surgical treatment, long term results and recurrence. J Cardiovasc Surg. 1993;34(1):49-53.

2Kassop D et al. Cardiac Masses on Cardiac CT: A Review. Current Cardiovascular Imaging Reports. 2014;7(8):9281. doi:10.1007/s12410-014-9281-1.

3Dato GMA et al.  Long term follow up of cardiac myxoma, (7-31years). Journal of Cardiovascular Surgery. 1993;34(2):114-43.

4Castells E et al. Cardiac myxoma: Surgical treatment, long term results and recurrence. J Cardiovasc Surg. 1993;34(1):49-53.

Image of the Month - February 2018 - An unusual cause of chest pain

An unusual cause of chest pain

Dylan Yong, Nadir Khan, Michelle Williams

Royal Infirmary of Edinburgh, University of Edinburgh

Case history

A 78-year-old female presented with chest pain of increasing frequency.  She had undergone investigation of chest pain with invasive coronary angiography and balloon angioplasty to her left coronary artery 2 weeks prior.

Admission chest x-ray showed a right sided pleural effusion and an enlarged cardiac contour (Figure 1A). Her troponin and D-dimer were raised. Electrocardiogram (ECG) was unremarkable.  She underwent a CT pulmonary angiogram (CTPA) which demonstrated a large pericardial effusion with maximum depth of 3.7cm (Figure 1B). Within this, there was an area of homogenous high attenuation in the epicardial space, following the course of right coronary artery. This was causing mass effect on the adjacent right atrium and ventricle. There was evidence of cardiac tamponade with straightening of the interventricular septum. Echocardiogram revealed a mass in the epicardial space suspicious for malignancy (Figure 1C).

A therapeutic pericardial drain was performed and haemoserous fluid obtained was sent for cytology. A total of 1.5 litres of fluid were drained from the right pleural space. Repeat CT post pericardial drainage demonstrated the mass more clearly (Figure 1D). Cytology from the pericardial effusion confirmed a diagnosis of primary cardiac diffuse large B cell lymphoma, stage IV. CT staging demonstrated no other sites of disease. Following discussion in a multi-disciplinary meeting, she received R-CHOP chemotherapy. She had an excellent response to the treatment, with a repeat 3-month interval CT scan demonstrating no measurable disease. (Figure 1E)

Discussion

Cardiac malignancy is rare, and primary cardiac lymphoma (PCL) accounts for only 1% of primary cardiac tumours.1 Amongst the small number of cases of PCL both B-cell and T-cell lymphomas have been reported.1 The majority of the PCL arise from the right side of the heart, particularly the right atrium.2 The reason for this distribution is unknown2. Cardiac involvement may also occur in up to 20% of patients who have diffuse nodal lymphoma.1 Lymphoma is an important differential diagnosis for cases when more than one cardiac chamber is involved.

Diagnosis of primary cardiac tumours is typically late and they are associated with a poor prognosis.2 Symptoms are related to the site of involvement of the heart. Ikeda et al. reviewed the presentations of 40 patients with PCL and found that patients generally complain of non-specific symptoms such as dyspnoea and oedema, or present with arrhythmias and pericardial effusion.3

Feb 2018 image of the month

Figure Legend

Figure 1A. Chest X-ray with an enlarged cardiac contour and a right pleural effusion.

Figure 1B. CTPA showing a right pleural effusion and pericardial effusion. Homogenous material surrounding the right coronary artery causing mass effect on the right atrium and right ventricle.

Figure 1C. Transthoracic echocardiography showing a mass suspicious for malignancy in the epicardial space.

Figure 1D. CT of the chest after pericardial drainage showing the mass in the epicardial space.

Figure 1E. CT of the chest after treatment showing no evidence of residual disease.

Multiple-choice questions

1. What percentage of patients with diffuse nodal lymphoma have cardiac involvement?

  1. 5%
  2. 10%
  3. 20%
  4. 30%
  5. 40%

2. What is the name given to the appearance of a pericardial effusion on chest x-ray?

  1. Sillhouette sign
  2. Chang sign
  3. Water-bottle sign
  4. Double bubble sign
  5. Snowman sign

3. Which chemotherapy drug is correctly associated with the type of cardiovascular toxicity?

  1. Anthracycline – heart failure
  2. Cyclophosphamide - ischaemia
  3. Fluorouracil – hypertension
  4. Cisplastin - ischaemia, thromboembolism
  5. Vincristine – heart failure

Answers: 1/C, 2/C, 3/A

References

  1. Patel J, Melly L, Sheppard MN. Primar cardiac lymphoma: B- and T-cell cases at a specialist UK centre. Ann Oncol 2010; 21(5):1041-5
  2. Ceresoli GL, Ferreri AJ, Bucci E, Ripa C, Ponzoni M, Villa E. Primary cardiac lymphoma in immunocompetent patients: diagnostic and therapeutic management. Cancer 1997;80: 1497-1506.
  3. Ikeda H, Nakamura S, Nishimaki H, Masuda K, Takeo T, Kasai K, et al. Primary lymphoma of the heart: case report and literature review. Pathol Int 2004;54(3):187-95

RCR/BSCI/BSCCT Travelling Cardiac Professor

The RCR and BSCI/BSCCT have established a travelling cardiac professor with the aims of highlight the role of imaging in the investigation of cardiovascular diseases, to promote best practice with regards to acquisition and reporting techniques, to promote cardiovascular imaging as a specialist interest in any NHS consultant appointment and to provide cardiovascular imaging teaching at both trainee and consultant level at centres that might not have access to such training opportunities. 

Planned visits from the RCR/BSCI/BSCCT travelling professor are as follows:

Dr Ed Nicol:

Mon 5 March – London 

Wed 14th March – S. Wales 

Mon 26th March Southampton 

Tues 22 May – Sheffield

Wed 23rd May – Hull

Mon 22-Wed 25th July 

Mon 10th Sept – Norwich 

 

Previous locations of visits from the RCR/BSCI/BSCCT traveling professor are as follows:

Dr Stephen Harden:

Chelsea and Westminster, London

Cardiff

Aberdeen

Leeds

St George's, London

Glasgow

Brighton

Oxford

 

BSCI/BSCCT members can login to the members section of the website to view reports from previous RCR/BSCI/BSCCT travelling cardiac professors.