Lutembacher syndrome with mitral valve calcification in a 31-year old male

J Pak Med Assoc. 2018 Feb;68(2):340-342.

Abstract

Lutembacher syndrome is characterized by a congenital ostium secundum atrial septal defect and an acquired mitral valve stenosis. We present a similar case in a 31-year old male who came in with orthopnoea, central cyanosis and pedal oedema. Examination revealed cardiac murmurs in tricuspid and apical regions. Chest x-ray showed signs of pulmonary congestion and ventricular enlargement. Electrocardiogaphy (ECG) revealed right axis deviation and right bundle branch block along with atrial fibrillation and Transthoracic Echocardiography (TTE) showed abnormal valves (mitral stenosis with calcification and tricuspid regurgitation) and dilated cardiac chambers. The patient was consequently treated with beta-blockers and diuretics and scheduled for valvular and septal repair via open heart surgery. The purpose of this case report is to assist cardiologists in diagnosing this syndrome accurately on the basis of symptoms and investigations.

Keywords: Atrial Septal Defect, Lutembacher Syndrome, Mitral stenosis, Transthoracic Echocardiography.

Publication types

  • Case Reports

MeSH terms

  • Adrenergic beta-Antagonists / therapeutic use
  • Adult
  • Amiloride / therapeutic use
  • Anticoagulants / therapeutic use
  • Atrial Fibrillation / diagnosis
  • Atrial Fibrillation / drug therapy
  • Atrial Fibrillation / etiology
  • Bundle-Branch Block / diagnosis
  • Bundle-Branch Block / etiology
  • Calcinosis / diagnostic imaging*
  • Calcinosis / physiopathology
  • Calcinosis / therapy
  • Cardiac Surgical Procedures
  • Cardiomegaly / diagnostic imaging
  • Cardiomegaly / etiology
  • Cyanosis / etiology
  • Diuretics / therapeutic use
  • Echocardiography
  • Edema / etiology
  • Electrocardiography
  • Foot
  • Furosemide / therapeutic use
  • Heart Septal Defects, Atrial / diagnostic imaging*
  • Heart Septal Defects, Atrial / physiopathology
  • Heart Septal Defects, Atrial / therapy
  • Humans
  • Lutembacher Syndrome / complications
  • Lutembacher Syndrome / diagnostic imaging*
  • Lutembacher Syndrome / physiopathology
  • Lutembacher Syndrome / therapy
  • Male
  • Mitral Valve Annuloplasty
  • Mitral Valve Stenosis / diagnostic imaging*
  • Mitral Valve Stenosis / physiopathology
  • Mitral Valve Stenosis / therapy
  • Tricuspid Valve Insufficiency / diagnostic imaging*
  • Tricuspid Valve Insufficiency / physiopathology
  • Tricuspid Valve Insufficiency / therapy
  • Warfarin / therapeutic use

Substances

  • Adrenergic beta-Antagonists
  • Anticoagulants
  • Diuretics
  • Warfarin
  • Amiloride
  • Furosemide