Clinical Scenario:
A 10-year-old boy with perinatally acquired HIV infection presents with progressive fatigue, reduced exercise tolerance and increasing breathlessness over 2 months. His antiretroviral therapy has been irregular. On examination, he is tachycardic and tachypneic, with a displaced apex beat, soft first heart sound and gallop rhythm. The liver is palpable 4 cm below the right costal margin.
Chest radiograph shows cardiomegaly with pulmonary venous congestion. Echocardiography shows global hypokinesia of the left ventricle with reduced systolic function.
There is no significant valvular abnormality or congenital heart defect.
Which of the following is the most likely diagnosis?
A. Viral myocarditis
B. HIV-associated cardiomyopathy
C. Rheumatic heart disease
D. Constrictive pericarditis
E. Hypertrophic cardiomyopathy
Correct answer & Explanation:
Explanation
Correct answer: B. HIV-associated cardiomyopathy
This child has perinatally acquired HIV with progressive congestive heart failure and global left ventricular systolic dysfunction, without a significant congenital or valvular lesion. This pattern is consistent with HIV-associated cardiomyopathy.
HIV-associated cardiac disease in children may have several mechanisms, including direct myocardial injury, immune-mediated myocardial damage, opportunistic infections, nutritional deficiencies and treatment-related factors. Cardiomyopathy may present with ventricular dysfunction and clinical features of heart failure.
The important clues in this case are:
- Long-standing HIV infection
- Poor adherence to antiretroviral therapy
- Progressive exercise intolerance and breathlessness
- Displaced apex beat and gallop rhythm
- Hepatomegaly from systemic venous congestion
- Cardiomegaly with pulmonary venous congestion
- Global rather than regional LV hypokinesia
- No significant congenital or valvular abnormality
Why not viral myocarditis? Viral myocarditis can produce global LV dysfunction and is an important differential. However, in this case, the established HIV infection with poor treatment adherence and the absence of another apparent cause make HIV-associated myocardial disease the most likely diagnosis.
Why not rheumatic heart disease? Rheumatic heart disease would be expected to produce characteristic valvular lesions rather than isolated global LV dysfunction.
Why not constrictive pericarditis? Constrictive pericarditis predominantly produces systemic venous congestion, but does not typically cause marked global LV systolic dysfunction.
Why not hypertrophic cardiomyopathy? Hypertrophic cardiomyopathy is characterized by increased ventricular wall thickness rather than a dilated, globally hypokinetic ventricle.
Reference
Lipshultz SE, Sleeper LA, et al. Cardiac effects of long-term antiretroviral therapy in children and adolescents with HIV infection. Pediatric HIV cardiovascular disease literature.
World Health Organization. Consolidated Guidelines on HIV Prevention, Testing, Treatment, Service Delivery and Monitoring.
