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Published on: May 5, 2018
Mid-aortic syndrome: a rare cause of heart failure in infants
Lu Zhao1, Li Zhu2, Qu-Ming Zhao1
1Cardiovascular Center, Children's Hospital of Fudan University, Shanghai, 201102, China.
Insights
Mid-aortic syndrome (MAS) can cause severe infant heart failure. Stent implantation may offer better outcomes than balloon angioplasty for this rare condition.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Vascular Surgery
Background:
- Mid-aortic syndrome (MAS) is a rare congenital condition characterized by narrowing of the aorta, potentially leading to severe hypertension and heart failure in infants.
- Early diagnosis of MAS is crucial but challenging, as symptoms can mimic other pediatric cardiac diseases like dilated cardiomyopathy.
- This report focuses on thoracoabdominal aortic coarctation, a severe form of MAS, in two infants.
Observation:
- Two infants, aged 2 and 11 months, presented with severe heart failure and were initially misdiagnosed.
- Diagnostic imaging was essential in confirming the correct diagnosis of critical thoracoabdominal aortic coarctation.
- Both patients underwent interventional procedures: one with balloon angioplasty alone, the other with balloon angioplasty and bare-metal stent implantation.
Findings:
- The patient treated solely with balloon angioplasty experienced a fatal outcome post-procedure.
- The infant who received balloon angioplasty with stent implantation demonstrated a positive recovery.
- Physical examination findings, including blood pressure differentials and pulse palpation, are vital for diagnosing unexplained infant heart failure.
Implications:
- Prompt and accurate diagnosis of MAS in infants is critical for timely intervention and improved prognosis.
- Stent implantation appears to be a potentially safer and more effective therapeutic strategy compared to balloon angioplasty alone in pediatric MAS cases.
- Heightened clinical suspicion and thorough physical examinations are paramount in identifying MAS in infants presenting with heart failure symptoms.
Abstract:
This case reports describe a rare disease, mid-aortic syndrome (MAS), that can cause severe heart failure and hypertension in infancy. The typical images, key points of diagnosis, and therapy methods of the disease have also been presented. We report two critical thoracoabdominal aortic coarctation cases in infants aged 2 and 11 months with severe heart failure. The patients were initially misdiagnosed as dilated myocardiopathy, with the correct diagnosis confirmed through imaging. Both patients underwent balloon angioplasty; one patient also had bare-metal stents implanted. The patient treated with balloon angioplasty alone died after the procedure, whereas the other patient recovered well. In conclusion, careful physical examinations, especially upper and lower extremity blood pressure differences and palpation of upper and lower limb pulses, are critical in unexplained infant heart failure cases. Stent implantation may be a safer and more effective treatment than simple balloon angioplasty in infants with MAS.
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