Clinical Scenario:
A 12-year-old boy presents with progressive abdominal distension and reduced exercise tolerance for 4 months. There is no history of jaundice or previous liver disease.
On examination, his blood pressure is 90/60 mmHg, pulse rate is 96/min, and the jugular venous pressure is elevated and rises with inspiration. He has marked ascites, hepatomegaly and bilateral ankle edema. Heart sounds are relatively quiet, and an early diastolic pericardial knock is audible.
Chest radiograph shows a normal-sized cardiac silhouette with pericardial calcification.
Which of the following is the most likely diagnosis?
A. Chronic liver disease
B. Dilated cardiomyopathy
C. Constrictive pericarditis
D. Nephrotic syndrome
E. Restrictive cardiomyopathy
Correct answer & Explanation:
Explanation
Correct answer: C. Constrictive pericarditis
This child has predominantly right-sided heart failure with systemic venous congestion, manifested by marked ascites, hepatomegaly, ankle edema and raised JVP.
The most important clinical clue is the JVP rising with inspiration (Kussmaul sign), which reflects impaired right ventricular filling. The early diastolic pericardial knock is another characteristic finding caused by abrupt cessation of ventricular filling by the rigid, non-compliant pericardium.
The low blood pressure (90/60 mmHg) and relatively narrow pulse pressure are compatible with reduced cardiac output, but they are supportive rather than diagnostic findings.
The normal-sized cardiac silhouette despite significant systemic venous congestion is useful in distinguishing constrictive pericarditis from dilated cardiomyopathy. Pericardial calcification, when present, is an additional clue to chronic constrictive pericardial disease, although its absence does not exclude the diagnosis.
In a child from a setting where tuberculosis is prevalent, tuberculous pericarditis is an important cause of constrictive pericarditis and should be considered when determining the etiology.
Why not restrictive cardiomyopathy? Both conditions can produce predominantly right-sided congestion and preserved systolic function. However, the pericardial knock and pericardial calcification strongly favor constrictive pericarditis.
Reference:
Yadav NK, Tivakaran S, Siddique MS. Constrictive Pericarditis. StatPearls. NCBI Bookshelf.
