Isolated right ventricular non-compaction in a newborn

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Highlights

  • Isolated right ventricular non - compaction is rare in newborns.

  • Isolated right ventricular non - compaction in a newborn may present with right heart failure.

  • Echocardiogram criteria for left ventricular non - compaction can be applied for isolated right ventricular non - compaction.

  • Cardiac magnetic resonance imaging is useful for making a diagnosis for isolated right ventricular non - compaction.

  • Diuretics and angiotensin converting enzyme inhibitors were useful in treating right heart failure in this newborn.

Abstract

Isolated right ventricular non - compaction is a rare congenital cardiomyopathy resulting from an arrest in normal endomyocardial embryogenesis. It is characterized by excessive prominent trabeculations and deep inter - trabecular recesses in the ventricular wall. The clinical syndrome includes systolic and diastolic dysfunction, heart failure, ventricular arrhythmias and cardioembolic events. Here we describe a newborn with isolated right ventricular non -compaction who had right heart failure and biventricular systolic dysfunction. Echocardiography and cardiac magnetic resonance imaging demonstrated the morphological abnormalities of the right ventricle.

Introduction

World Health Organization/International and Federation of Cardiology Task Force categorized non- compaction of the ventricular myocardium as an “unclassified cardiomyopathy” in 1995. American Heart Association in its scientific statement in 2006 classified it under the “genetic” section of “primary cardiomyopathies (1). Its prevalence is 0.14% in the paediatric population with a male to female ratio of 5.7 to 1.2. The usual site of involvement is the left ventricle. Involvement of both ventricles or isolated non - compaction of the right ventricle has been described rarely (3).

Section snippets

Case Report

A baby girl was admitted to the neonatal intensive care unit with cyanosis and respiratory distress at day 3 of life. She was delivered via spontaneous vertex delivery at 37 weeks with good Apgar scores and a birth weight of 3.12 kg. Her foetal echocardiogram revealed dilated right atrium and ventricle with mild tricuspid regurgitation. There was no family history of congenital heart disease. She was not dysmorphic, oxygen saturation was 85%–88% on room air, heart rate of 150 beats per minute

Discussion

During normal foetal development, ventricles initially are a meshwork of interwoven fibres and between 5th and 8th week, ventricular compaction occurs from the base towards the apex and from epicardium to endocardium. During compaction, inter-trabecular spaces in ventricular myocardium are obliterated and the recesses in the trabecular network are reduced to capillaries. Failure of this endocardial morphogenesis and regression of ventricular sinusoids results in ventricular non - compaction.

Conclusion

Knowledge and understanding about aetiology, embryogenesis of the myocardium, genetic background, diagnosis and outcome of left ventricular non - compaction have steadily improved. However, data for right ventricle non – compaction are lacking due to few case reports and under diagnosis of the disease. It is important to consider this rare cardiomyopathy as a differential in a neonate presenting with right heart failure.

Declarations of Interest

Authors declare there are no conflicts.

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