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To Be or Not to Be Eisenmenger: The Different Shades of Blue

Sarit S Cohen1, Sathiji Kathiresu Nageshwaran2, Raghav Murthy3

  • 1Department of Cardiology, Montefiore-Einstein Center for Heart and Vascular Care, Bronx, New York, USA.

JACC. Case Reports
|July 28, 2021
PubMed

Insights

Eisenmenger syndrome is a severe complication of congenital heart disease. This case highlights a patient initially presumed to have this condition who actually had a treatable defect, leading to successful repair.

Area of Science:

  • Cardiology
  • Congenital Heart Disease
  • Pulmonary Hypertension

Background:

  • Eisenmenger syndrome is defined by untreated congenital heart defects leading to pulmonary hypertension, shunt reversal, and cyanosis.
  • Patients with presumed Eisenmenger syndrome are often deemed inoperable due to the severity of their condition.

Observation:

  • A 40-year-old cyanotic patient with congenital heart disease was diagnosed with presumed Eisenmenger syndrome.
  • The patient was considered for inoperable treatment due to the presumed diagnosis.

Findings:

  • Diagnostic testing revealed a partial atrioventricular septal defect.
  • Crucially, there was no evidence of pulmonary arterial hypertension, contradicting the Eisenmenger syndrome diagnosis.
  • The patient underwent successful surgical repair of the atrioventricular septal defect.

Implications:

  • This case underscores the importance of accurate diagnosis in congenital heart disease, even in complex presentations.
  • Misdiagnosis of Eisenmenger syndrome can lead to missed opportunities for life-saving interventions.
  • Successful surgical repair of previously inoperable congenital defects is possible with precise diagnostic evaluation.

Keywords:
ASD, atrial septal defectES, Eisenmenger syndromeEisenmenger syndromeLA, left atriumLV, left ventriclePA, pulmonary arteryPVR, pulmonary vascular resistanceRA, right atriumRV, right ventricleSVC, superior vena cavacongenital heart diseasepulmonary hypertension

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