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Palliative Mitral Valve Repair During Infancy for Neonatal Marfan Syndrome

Hiroto Kitahara1, Ryo Aeba1, Hidenobu Takaki1

  • 1Division of Cardiovascular Surgery, Keio University, Tokyo, Japan.

The Annals of Thoracic Surgery
|April 24, 2016
PubMed

Insights

Neonatal Marfan syndrome (nMFS) infants rarely survive infancy. This case demonstrates successful mitral valve repair in an nMFS patient, achieving long-term survival past age 11.

Area of Science:

  • Pediatric Cardiology
  • Medical Genetics
  • Congenital Heart Disease

Background:

  • Neonatal Marfan syndrome (nMFS) is a severe connective tissue disorder with high infant mortality.
  • Congestive heart failure due to mitral valve insufficiency is a common presentation in nMFS.
  • Surgical intervention for severe mitral valve issues in nMFS is challenging due to the condition's systemic effects.

Observation:

  • An infant diagnosed with nMFS presented with severe congestive heart failure and mitral valve insufficiency.
  • The patient underwent two palliative mitral valvuloplasty procedures before a definitive mitral valve repair.
  • An annuloplasty ring was intentionally omitted during the initial repair stages.

Findings:

  • The patient's mitral valve function remained within normal limits post-definitive repair.
  • The patient survived until the age of 11 years with sustained normal mitral valve function.
  • This represents the longest documented survival following mitral valve repair in a patient with nMFS.

Implications:

  • This case highlights the potential for long-term survival in nMFS patients with complex congenital heart disease.
  • It suggests that a tailored surgical approach, including staged mitral valve repair, can be effective.
  • Further research into long-term outcomes and management strategies for nMFS is warranted.

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