Clinical characteristics and short-term outcomes of left ventricular non-compaction cardiomyopathy in neonates

TL;DR

This study examines the clinical features and outcomes of left ventricular non-compaction cardiomyopathy in newborns, finding varied symptoms and some signs of recovery with treatment.

Contribution

The study provides new insights into the clinical variability and short-term outcomes of neonatal left ventricular non-compaction cardiomyopathy.

Findings

Neonatal LVNC presents with a wide range of symptoms, from asymptomatic to severe heart failure.

Heart failure at diagnosis is linked to worse outcomes, but some neonates showed improved heart function with treatment.

Two neonates died, while five asymptomatic survivors had normal heart function after one year.

Abstract

Left ventricular non-compaction (LVNC) is a rare type of cardiomyopathy. It is more difficult to diagnose during the neonatal period. This study reported the clinical manifestations of neonatal LVNC at initial diagnosis and investigated their short-term outcomes.

A cohort of 10 neonates was enrolled. Their clinical characteristics were analyzed, and follow-up was conducted for 1 year.

The neonates had an average gestational age of 35.82 weeks and an average birth weight of 2,636 g. The average age at initial diagnosis was 12 days and clinical manifestations were highly variable, ranging from asymptomatic (20%) to cyanosis (50%), dyspnea (50%), arrhythmia (60%), and heart failure (30%). Plasma myocardial injury markers were elevated, and electrocardiogram abnormalities were present in 90% of infants. Echocardiography revealed an average left ventricular (LV) non-compacted/compacted ratio of 2.96 and reduced systolic function (ejection fraction: 38.5%; fractional shortening: 18.8%). The average LV internal diameters were both above the normative reference intervals, with an end-diastolic diameter of 2.26 cm and an end-systolic diameter of 1.84 cm. During follow-up, two neonates died. Among the eight survivors, three with non-isolated LVNC underwent cardiac surgery, whereas five were asymptomatic with normal LV systolic function at 1 year of age.

The clinical presentation of neonatal LVNC varies widely, from asymptomatic to those with dyspnea, cyanosis, arrhythmia, or heart failure. In our cohort, heart failure at initial diagnosis was associated with a poorer prognosis. Short-term follow-up suggested that with aggressive management, myocardial function may show signs of reversibility in a subset of neonates.

Figures