Pericardial Disease Viva

Long Case Viva Defence

Candidate's opening statement (model answer)

"Mr Thompson is a 62-year-old retired schoolteacher who presents with a 6-month history of progressive exertional dyspnoea, bilateral leg swelling, and abdominal distension. He has a background of Hodgkin lymphoma treated with mediastinal radiotherapy and ABVD chemotherapy 20 years ago, type 2 diabetes, and hypertension. ^[1]

His past history also includes aortic valve replacement (bioprosthetic) 5 years ago for radiation-induced aortic stenosis, and permanent atrial fibrillation. ^[1]

On examination, his JVP is elevated to 10 cm above the sternal angle with a prominent Y descent that rises further on inspiration — a Kussmaul sign. A high-pitched early diastolic sound is audible at the left lower sternal border — a pericardial knock. He has hepatomegaly 4 cm below the costal margin, moderate ascites, and pitting oedema to the knees bilaterally. ^[1]

His echocardiogram shows an ejection fraction of 58%, thickened pericardium, a diastolic septal bounce, preserved medial e-prime of 12 cm/s, and expiratory diastolic flow reversal in the hepatic veins. CT confirms pericardial thickness of 4 mm. Cardiac MRI shows pericardial late gadolinium enhancement but no myocardial LGE. BNP is 140. ^[1]

His main problems are:

1. Constrictive pericarditis, radiation-induced, with an inflammatory (possibly transient) component given the pericardial LGE
2. Radiation-induced valvular disease — prior bioprosthetic AVR (needs surveillance for degeneration)
3. Radiation-induced coronary artery disease — needs assessment
4. Permanent atrial fibrillation — on apixaban
5. Type 2 diabetes and hypertension
6. Chronic right heart failure with cardiac cirrhosis risk
7. Polypharmacy and complex medication management ^[1]

My working diagnosis is constrictive pericarditis secondary to mediastinal radiotherapy, with the possibility of a transient inflammatory component. I need to differentiate this from radiation-induced restrictive cardiomyopathy, which can coexist. My priority is confirming the diagnosis with the echo and MRI findings, trialling a period of anti-inflammatory therapy given the pericardial LGE, and planning for pericardiectomy if the constriction is fixed." ^[1]

Examiner probing questions and model answers

Q1: "How confident are you that this is constriction and not restriction, given the radiation history?" ^[1]

"My confidence is moderate to high but not absolute, and that is the key issue. Five features favour constriction: the preserved medial e-prime of 12 cm/s — the single best non-invasive discriminator, because it shows the myocardium is normal and the restriction is in the pericardium; the diastolic septal bounce on echo, reflecting ventricular interdependence; the expiratory hepatic vein flow reversal, also specific for constriction; the pericardial thickness of 4 mm on CT; and the cardiac MRI showing pericardial late gadolinium enhancement with no myocardial LGE — if this were restrictive cardiomyopathy I would expect myocardial LGE, such as subendocardial circumferential enhancement for amyloid. ^[1]

However, I acknowledge that radiation damages both the pericardium AND the myocardium, so a mixed picture is possible. The preserved e-prime and the absence of myocardial LGE are reassuring that the myocardium is not the dominant problem. If the patient were to fail pericardiectomy or show clear myocardial disease on further imaging, I would reconsider a mixed constriction-restriction physiology." ^[1]

Q2: "What is the significance of the pericardial late gadolinium enhancement on the MRI?" ^[1]

"Pericardial late gadolinium enhancement indicates active pericardial inflammation, not just chronic fibrosis. This raises the possibility of transient — or reversible — constriction. Studies show that up to 20 percent of constrictive presentations are transient and can resolve with anti-inflammatory therapy (NSAIDs or colchicine, sometimes a short steroid taper) over 2 to 3 months. Given this patient has pericardial LGE and a relatively recent symptom onset of 6 months, I would trial a period of anti-inflammatory therapy before committing to pericardiectomy — specifically colchicine 0.5 mg twice daily, with clinical and echo reassessment at 3 months. If the constrictive physiology resolves, surgery is avoided." ^[1]

Q3: "Would you perform right and left heart catheterisation?" ^[1]

"Yes — cardiac catheterisation provides haemodynamic confirmation and is particularly valuable when non-invasive imaging is equivocal or when mixed physiology is suspected. In constrictive pericarditis I expect: equalisation of LV and RV diastolic pressures (within 5 mmHg); the dip-and-plateau or square-root sign on the ventricular pressure trace (rapid early diastolic filling followed by an abrupt plateau as the ventricle hits the rigid pericardium); an RVEDP to RV systolic pressure ratio over one-third; and a PA systolic pressure usually under 55 mmHg. If the PA systolic is markedly elevated or the LV diastolic pressure significantly exceeds RV, that points toward restrictive cardiomyopathy or mixed disease. In this patient, with prior valve surgery and radiation, catheterisation would also allow coronary angiography to assess for radiation-induced coronary disease." ^[1]

Q4: "The patient has a bioprosthetic aortic valve replaced 5 years ago. How does this complicate pericardiectomy?" ^[1]

"A prior sternotomy and mediastinal radiotherapy both make pericardiectomy technically more challenging and higher risk. Repeat sternotomy carries risk of injury to adherent structures, particularly the right ventricle and prior grafts if CABG was done. The radiation causes dense fibrosis and adhesions in the mediastinum and may have thinned the right ventricular free wall. Perioperative mortality for pericardiectomy is 5 to 15 percent overall but is higher — up to 15 to 20 percent — in irradiated and reoperative patients. The case must go to a specialist cardiothoracic centre with experience in complex redo pericardiectomy. The bioprosthetic AVR also needs assessment for degeneration, as bioprosthetic valves last 10 to 15 years on average — at 5 years it is probably still functioning, but I would assess the gradients on echo." ^[1]

Q5: "How would you manage the right heart failure symptoms while awaiting the trial of medical therapy or surgery?" ^[1]

"Cautious diuresis — frusemide 20 to 40 mg daily or spironolactone 12.5 to 25 mg daily for the ascites and oedema. The key principle is to avoid over-diuresis: these patients are preload-dependent because the rigid pericardium prevents ventricular filling, and aggressive diuresis can precipitate hypotension and renal failure. I would aim for symptom relief and a modest weight reduction, monitoring renal function and blood pressure closely. I would also restrict dietary sodium and fluid if the ascites is problematic." ^[1]

Q6: "What is the patient's prognosis and how would you discuss it?" ^[1]

"The prognosis depends on whether the constriction is transient or fixed. If transient and responsive to anti-inflammatory therapy, the outlook is favourable with medical management. If fixed and requiring pericardiectomy, the 5-year survival after surgery is 70 to 80 percent in well-selected patients, but is worse in irradiated patients because of the comorbid myocardial, coronary, and valvular damage. The patient needs to understand that pericardiectomy is major surgery with significant risk, that the decision involves balancing this risk against the progressive nature of untreated constriction, and that a multidisciplinary approach at a specialist centre is essential. I would discuss the possibility of a mixed constriction-restriction picture — in which case surgery may not fully resolve symptoms — honestly but constructively. Shared decision-making with the patient and cardiothoracic team is central." ^[1]