Table 1.

VLCADD patients' natural history.

Study	Age at presentation	Clinical manifestation	Cardiac presentation	Diagnosis	Outcome
Dereddy NR	2 months	Respiratory distress, failure to thrive, hepatomegaly, hypotonia	Left ventricular hypertrophy and dilatation, reduced systolic function and pericardial effusion	Skin fibroblasts enzyme activity	Normal cardiac function after 8 years follow-up
	4 months	Metabolic acidosis, cardiogenic shock	Left ventricular dilatation with reduced systolic function	Gene mutation	Acceptable cardiac size and function after 4 years follow-up
Katz S	2 days	Lethargy, increased CK and liver transaminases	First: small ASD After 6 months: Massive pericardial effusion, left ventricular hypertrophy and systolic dysfunction requiring ECMO	Plasma acyl carnitine profile, gene mutation	Death
Mathur A∗	1day to 22 months	a variety of presentations	Dilated and hypertrophic cardiomyopathy	Gene mutation	Normal systolic function in 6
Sharef SW	Immediate neonatal period	Neonatal hypoglycemia, positive family history	Dilated and hypertrophic left ventricle, reduced ejection fraction, pericardial effusion	Acylcarnitine profile, skin fibroblast enzyme activity	Normal cardiac function after 4 years follow-up

^∗This study consisted of 18 genetically documented VLCADD cases.