An 18-year-old woman with truncus arteriosus presented with septic shock due to methicillin-sensitive Staphylococcus aureus infective endocarditis of her right-ventricle-to-pulmonary-artery (RV-PA) conduit requiring urgent conduit replacement and a prolonged admission complicated by acute respiratory distress syndrome and significant deconditioning. She had three separate presentations to external medical practitioners in the week preceding admission. This case underscores the complexity of managing infective endocarditis in patients with congenital heart disease and the importance of early referral to a specialist congenital centre.
Case presentation
An 18-year-old woman presented with a three-week history of subjective fevers, nausea and anorexia. Background history included congenital heart disease (CHD) with truncus arteriosus requiring right-ventricle-to-pulmonary-artery (RV-PA) conduit replacements, most recently with a 25-mm ‘Freestyle’ porcine conduit 10 years prior. She was not previously known to our centre. She had no regular medications and no recreational drug use.
She originally presented with her three-week history to her general practitioner then twice to a peripheral hospital emergency department (ED) within one week. At all encounters, she was documented to have normal vital signs, white cell count (WCC) and C-reactive protein (CRP) levels. She was subsequently reassurance and discharged. No blood cultures were taken. She re-presented a third time to the external ED one week later with dyspnoea (now week four of symptoms). She was haemodynamically unstable with a heart rate of 110 beats per minute, hypotensive with a blood pressure of 90/60 mmHg, tachypnoeic with a respiratory rate of 26 breaths per minute, and febrile with a body temperature of 39.5°C. She had an elevated WCC of 15.3×109/L (reference range: 4–12×109/L), elevated CRP of 180 mg/L (reference cut-off value: <5 mg/L), and increased lactate of 4 mmol/L (reference cut-off value: <1.8 mmol/L), indicating systemic inflammation and poor end-organ perfusion. Urgent computed tomography of the pulmonary arteries (CTPA) reported no pulmonary embolism and changes around her RV-PA conduit thought to be consistent with expected post-operative changes. She was admitted to the intensive care unit (ICU) with septic shock. Blood cultures before the administration of empirical intravenous ceftriaxone and flucloxacillin were positive for methicillin-sensitive Staphylococcus aureus (MSSA). She was discussed with the quaternary hospital adult CHD (ACHD) service and urgently transferred to our ICU on day 3 of admission.
Urgent transthoracic echocardiography (TTE) performed on the day of transfer demonstrated vegetations on the RV-PA conduit with possible contained rupture and new significant mixed conduit dysfunction associated with mild RV dilatation and moderate dysfunction (Figure 1A–C). A diagnosis of MSSA infective endocarditis (IE) of the RV-PA conduit was made without the need for transoesophageal echocardiography. Retrospective review of the external CTPA was suspicious for contained rupture of the RV-PA conduit (Figure 1D).
![Lankaputhra - Figure 1. Pre-operative transthoracic echocardiogram (A) of the right-ventricle-to-pulmonary-artery (RV-PA) conduit (yellow arrow) with colour flow Doppler demonstrating significant flow acceleration and regurgitation across the narrowed valve and communication with a probable abscess (white arrow). Continuous wave Doppler profile confirms significant pulmonary valve stenosis with elevated gradient (peak gradient: 72 mmHg; reference cut-off: <36 mmHg) (B) and significant regurgitation (short pressure half time: 73 ms [severe <100 ms]; dense jet with early diastolic flow termination) (C) across the valve. D: Computed tomography of the pulmonary arteries demonstrating the RV-PA conduit (yellow arrow) with significant calcification and outpouching consistent with abscess formation (white arrow).](https://bjcardio.co.uk/wp-content/uploads/2026/08/Lankaputhra-FIgure-1.jpg)
A multidisciplinary ‘heart team’ consensus involving cardiology, cardiothoracic surgery, infectious diseases and ICU teams was made for urgent surgical RV-PA conduit replacement the following day. Operative findings confirmed conduit destruction with abscess formation requiring replacement with a 25-mm pulmonary allograft, and a mediastinal abscess requiring drainage.
TTE on post-operative day four revealed normal RV size with mild systolic dysfunction. A complicated post-operative course ensued, with acute respiratory distress syndrome and ventilator-associated pneumonia requiring prolonged respiratory support and a surgical tracheostomy on day 12. She also experienced significant deconditioning with a 20 kg weight loss to 38 kg requiring intensive rehabilitation and dietitian input. She was discharged home on day 52 and was well at five months post-discharge.
Discussion
The ACHD population is rapidly growing as children with CHD survive into adulthood. In Australia alone, it is predicted that 1 in 150 young adults will have some form of ACHD within the next decade.1 CHD patients are at 20–70-fold higher risk of developing IE, particularly with intracardiac prosthetic material and MSSA.2 MSSA is a highly virulent organism with a propensity for rapid tissue invasion and abscess formation, which may explain the fulminant course and rapid conduit destruction in this case. The presentation of IE in young ACHD patients is often non-specific with fever and general non-wellbeing the most common symptoms, and a longer time between symptom onset and hospital admission than in non-CHD patients.2 Young patients often compensate very well and do not show clinical deterioration until late in the disease process.2,3 Therefore, a high index of suspicion and aggressive management, including early referral to a specialised congenital centre, is crucial as delayed diagnosis predicts adverse outcomes.3 This includes a low threshold to perform blood cultures in any setting, particularly in those with prosthetic material.
This case highlights the importance of effective communication and collaboration between general practitioners and non-congenital and congenital referral centres in managing complex CHD. The delayed diagnosis and referral of this patient, despite her repeated visits, highlight the need for increased education of IE in the ACHD population and improved coordination of care between healthcare settings.1,3–5 A delay in referral can have deleterious consequences, as evidenced in this case by the rapid deterioration of the patient’s condition and eventual need for emergent, complex surgical interventions.
The ‘heart team’ multidisciplinary approach involving cardiologists, cardiac surgeons and other health professionals is well recognised in ACHD management.5 However, this collaborative approach should also extend to involve non-congenital centres to foster a closer relationship, with clear communication pathways and protocols. Regular meetings involving both non-congenital and congenital centres can facilitate discussion of complex cases, streamline referrals, and ensure prompt and appropriate management.5 This may be facilitated by telemedicine to allow for real-time case discussion and sharing of diagnostic results.5
Our case highlights the importance of a high index of suspicion for IE in ACHD patients, particularly in those with prosthetic material, and early referral and discussion with a specialist congenital centre.
Key messages
- All medical practitioners managing patients with complex congenital heart conditions should maintain a high index of suspicion for infective endocarditis (IE) with a low threshold to perform blood cultures
- Delayed diagnosis and management of IE in congenital heart disease patients, particularly in those with prosthetic material, can result in significant complications even after urgent surgical intervention
- Early referral to a specialised congenital centre is crucial for comprehensive management of potential IE, to expedite diagnosis and timely surgical intervention if required
Conflicts of interest
None declared.
Funding
None.
Patient consent
Written informed consent was obtained from the patient for the publication of this case report and any accompanying images.
Ethical approval
Ethical approval was not required for this case report in accordance with guidelines from The Royal Melbourne Hospital, as it describes the clinical course of a single patient from whom written informed consent for publication was obtained.
References
1. Nicolae M, Gentles T, Strange G et al. Adult congenital heart disease in Australia and New Zealand: a call for optimal care. Heart Lung Circ 2019;28:521–9. https://doi.org/10.1016/j.hlc.2018.10.015
2. van Melle JP, Roos-Hesselink JW, Bansal M et al. Infective endocarditis in adult patients with congenital heart disease. Int J Cardiol 2023;370:178–85. https://doi.org/10.1016/j.ijcard.2022.10.136
3. Yoshinaga M, Niwa K, Niwa A et al. Risk factors for in-hospital mortality during infective endocarditis in patients with congenital heart disease. Am J Cardiol 2008;101:114–8. https://doi.org/10.1016/j.amjcard.2007.07.054
4. Abegaz TM, Bhagavathula AS, Gebreyohannes EA, Mekonnen AB, Abebe TB. Short-and long-term outcomes in infective endocarditis patients: a systematic review and meta-analysis. BMC Cardiovasc Disord 2017;17:291. https://doi.org/10.1186/s12872-017-0729-5
5. Warnes CA, Liberthson R, Danielson GK et al. Task force 1: the changing profile of congenital heart disease in adult life. J Am Coll Cardiol 2001;37:1170–5. https://doi.org/10.1016/s0735-1097(01)01272-4
