A Child With Spontaneous Resolution Of Idiopathic Mid-aortic Syndrome Presented With Dilated Cardiomyopathy
Kim, B. J., Kim, G. B., & Lee, S. Y. (2024). A child with spontaneous resolution of idiopathic mid-aortic syndrome presented with dilated cardiomyopathy. Cardiology in the young, 34(11), 2477–2479. https://doi.org/10.1017/S1047951124036357
View Original Source →Abstract
Mid-aortic syndrome is an uncommon vascular disease characterised by lower thoracic and upper abdominal aorta stenosis and can occur even in neonatal or infant periods. Here, we report an interesting case of a 2-month-old female with diffuse hypoplasia of the lower abdominal aorta and secondary dilated cardiomyopathy. In our patient, her abdominal aortic narrowing spontaneously normalised over time with the administration of consistent and goal-directed heart failure therapy, supporting adequate growth and natural recovery.
Case Details
Disease Location
Aorta (abdominal)
Personal Characteristics
2-month-old female
Clinical Characteristics
Newly confirmed dilated left ventricle due to symptoms of respiratory distress and cough was transferred to our paediatric icu for further treatment. Echocardiography showed that her left ventricle was dilated up to 33.3 mm and left ventricle ejection fraction was measured at 10% despite continuous administration of inotropic agents including dopamine (5 mcg/kg/min) and dobutamine (5 mcg/kg/min). Laboratory showed elevated lactate and serum b-type natriuretic peptide levels. Oral digoxin (5 mcg/kg/dose every 12 hours) and diuretics (furosemide: 1 mg/kg/day, spironolactone: 1 mg/kg/day), angiotensin-converting enzyme inhibitor (captopril 0.3 mg/kg/dose every 8 hours) and oral beta-blocker (carvedilol 0.04 mg/kg/dose every 12 hours) were started. Despite her decreased cardiac contractility, she frequently exhibited uncontrolled elevated blood pressure, reaching up to 125/72 mmhg. CT angiography of the aorta was performed, which showed a diffusely small caliber of the abdominal aorta and its branches with no evidence of coarctation of the proximal descending thoracic aorta. Laboratory test revealed hyperreninemic hyperaldosteronism and elevated aldosterone. Diagnosis of secondary hypertension possibly resulting from idiopathic mid-aortic syndrome was made. Genetic testing revealed a novel unclassified variant of tmpo gene. At 2 years of age, digoxin, furosemide, and spironolactone were suspended when her cardiac function was assessed at 51.6%
Remission Characteristics
Eight years after diagnosis, a follow-up CT angiography of the aorta showed an interval increase in the diameter of the abdominal aorta and slightly small-sized renal arteries with no focal stenosis
Treatment & Mechanisms
Proposed Remission Mechanisms
Growth and natural recovery
Clinical Treatment
Dopamine, dobutamine digoxin, furosemide, spironolactone, captopril, caverdilol.