# Isolated pulmonary valve infective endocarditis in isolated congenital supravalvular pulmonary stenosis: a case report

## TL;DR

A rare case of pulmonary valve infective endocarditis in a patient with congenital heart disease highlights the challenges in diagnosis and treatment.

## Contribution

This case report adds to the limited literature on isolated pulmonary valve infective endocarditis in the context of supravalvular pulmonary stenosis.

## Key findings

- Transthoracic echocardiography had limited sensitivity in diagnosing the condition, requiring transesophageal echocardiography for confirmation.
- The patient required surgical intervention for valve replacement and showed good recovery post-treatment.
- The case emphasizes the importance of high clinical suspicion for rare but life-threatening conditions in patients with congenital heart disease.

## Abstract

Isolated pulmonary valve stenosis, of which supravalvular pulmonary stenosis is a subcategory, rarely occurs in live births. Isolated pulmonary valve infective endocarditis is uncommon and typically arises in settings of recurrent transient bacteremia, immunodeficiency, or congenital heart disease. Any infective endocarditis carries high morbidity with complications commonly including congestive heart failure, acute renal failure, and septic emboli among others. Consistent with previously published cases of pulmonary valve infective endocarditis, these cases can be challenging to diagnose and treat.

We present the case of a 52-year-old male with well-controlled human immunodeficiency virus on antiviral therapy and known non-syndromic congenital supravalvular pulmonary stenosis who developed pulmonary valve infective endocarditis. He originally presented to the emergency room with acute delirium and was diagnosed with Streptococcus agalactiae meningitis and bacteremia due to suspected pharyngitis, but despite antibiotics, progressed to meningoencephalitis and septic shock. His transthoracic echocardiogram was without evidence of infective endocarditis; however, a transesophageal echocardiogram was performed due to a high index of suspicion and confirmed the diagnosis. The patient subsequently required right ventricular outflow tract pericardial patch repair and replacement of the pulmonary valve with a bioprosthetic valve. At follow-up, he was asymptomatic and tolerating rehabilitation.

Pulmonary valve infective endocarditis can be difficult to diagnose because of limited sensitivity on transthoracic echocardiography and often underappreciated predisposing factors such as simple congenital heart disease. This case provides a reminder to providers of a rare, life-threatening entity that requires a high index of suspicion to diagnose.