Bronchiolitis Unveiling a Hidden Heart: A Case of Left Ventricular Non-compaction

Aishwarya Padubidri Muralidhar.

Paediatric Cardiac ICU, CARE Hospitals, Banjara Hills, Hyderabad.

ADDRESS FOR CORRESPONDENCE
Aishwarya Padubidri Muralidhar, Paediatric Cardiac ICU, CARE Hospitals, Banjara hills road no 1, Hyderabad.
Email: aishu.padubidri.m@gmail.com

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Keywords
Bronchiolitis, left ventricular non compaction, pediatric cardiology

Clinical Problem
An 8-month-old male infant presented with a three-day history of cough, rhinorrhoea, and low-grade fever and one day history of fast breathing and feeding difficulties. He was born full-term with no significant past medical history. Physical examination revealed an irritable child with tachypnoea, tachycardia, subcostal, intercostal retractions, and bilateral wheezes. Oxygen saturation was 92% on room air. Other systemic examinations were normal. Complete blood count was unremarkable. C-reactive protein was mildly elevated. Chest X-ray showed hyperinflation and patchy atelectasis consistent with bronchiolitis. The infant was managed conservatively with supportive care, including supplemental oxygen, nebulized bronchodilators, and adequate hydration. However, despite initial improvement, the child continued to exhibit tachypnoea and tachycardia. Due to the persistent symptoms, a transthoracic echocardiogram was performed which revealed a left ventricle with prominent trabeculations and deep intertrabecular recesses, raising suspicion for Left ventricular non compaction (LVNC). Left ventricular systolic function was reduced with an estimated ejection fraction of 45%.
A detailed cardiac evaluation, including a repeat echocardiogram and electrocardiogram (ECG), was performed. The repeat echocardiogram confirmed the presence of prominent trabeculations and deep intertrabecular recesses in the left ventricle, consistent with LVNC. ECG was unremarkable. Cardiac magnetic resonance imaging (CMR) was performed to further characterize the LVNC and assess for any associated abnormalities. CMR confirmed the diagnosis of LVNC with moderate severity.
On consultation with the paediatric cardiologist, the infant was started on diuretics and an angiotensin-converting enzyme inhibitor to manage heart failure symptoms associated with LVNC to which the child responded well and was discharged on day 7 of admission. The infant is currently asymptomatic and receiving regular cardiology follow-up.
This case highlights the importance of considering underlying cardiac conditions in infants with persistent respiratory symptoms following an episode of bronchiolitis. A comprehensive evaluation, including careful clinical assessment, thorough investigations, and close follow-up, in infants with suspected cardiac involvement can aid in early diagnosis and prompt treatment.

What is left ventricular non compaction?

Discussion
Left Ventricular Noncompaction (LVNC) is a rare primary cardiomyopathy with a likely genetic origin. It primarily affects the left ventricle, characterized by a distinct two-layered myocardial structure. An abnormally trabeculated, thick, and spongy inner layer communicates with the ventricular cavity, while a thin compacted outer layer forms the exterior. LVNC may occur in isolation or be associated with other cardiomyopathies, congenital heart diseases, neuromuscular disorders, or reversible conditions like pregnancy, athletic activity, and hemoglobinopathies.¹
The prevalence of LVNC ranges from 0.014% to 0.26%, with a higher incidence in males.² Clinical presentations can vary widely and may include asymptomatic cases, cardiac failure symptoms (dyspnoea, exercise intolerance, peripheral oedema), arrhythmias, thromboembolic events, and sudden cardiac death.³

Diagnosis
Two-dimensional echocardiography (2D-ECHO) is the initial diagnostic tool. A ratio of the trabeculated spongy layer to the compact layer exceeding 2 in end-systole on short-axis views is a key diagnostic criterion. 2D-ECHO also helps rule out associated congenital heart defects. Cardiac magnetic resonance imaging (CMR) can confirm the diagnosis and provide additional information, such as ventricular thrombi and myocardial fibrosis.⁴

Management
Management is primarily supportive. Close monitoring is crucial for patients with normal ventricular function to detect complications. Aspirin or oral anticoagulants is recommended for patients with reduced ventricular function due to the increased risk of clot formation associated with hype trabeculations. Anticoagulants is also advised for LVNC patients with atrial fibrillation, as these individuals are at higher risk of systolic dysfunction over time. Other arrhythmias should be managed according to standard protocols including implantable cardiac defibrillator in patients with severe arrhythmias to prevent sudden death. Patients with congestive heart failure may benefit from diuretics, angiotensin-converting enzyme inhibitors (ACE inhibitors), or angiotensin receptor blockers (ARBs). Beta-blockers may be necessary in cases with concomitant hypertrophic cardiomyopathy. Approximately 12% of LVNC patients may progress to end-stage disease requiring cardiac transplantation.⁵

Prognosis
The prognosis of children with LVNC is variable. However, a significant proportion are likely to develop systolic dysfunction over time. Mortality rates are influenced by factors such as ejection fraction and the presence of complications like arrhythmias and thromboembolic events.⁶

Compliance with ethical standards

Funding: None

Conflict of Interest: None

References

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DOI: https://doi.org/10.7199/ped.oncall.2025.60

Cite this article as:
Muralidhar A P. Bronchiolitis Unveiling a Hidden Heart: A Case of Left Ventricular Non-compaction. Pediatr Oncall J. 2025 Feb 01. doi: 10.7199/ped.oncall.2025.60