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KEY FEATURES

ESSENTIALS OF DIAGNOSIS

  • Congenital atrial septal defect plus rheumatic mitral stenosis

GENERAL CONSIDERATIONS

  • Atrial septal defect (ASD) is a common congenital heart disease that often escapes notice in early childhood if small

  • In parts of the world where rheumatic heart disease is also common, occasionally ASD and mitral stenosis occur together (Lutembacher’s syndrome)

  • What makes this coincidence of special importance is the physiologic interaction between the 2 conditions

  • Mitral stenosis increases the pressure in the left atrium and increases the left-to-right shunt at the atrial level

  • An ASD allows some decompression of left atrial pressure at the expense of reduced transatrial flow into the left ventricle

  • Iatrogenic Lutembacher’s syndrome occurs when balloon mitral valvuloplasty for mitral stenosis is attempted by the transatrial septal approach and stenosis relief is incomplete

    • – The persistently high left atrial pressure in this situation prevents atrial septal healing and results in a persistent ASD

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

  • Fatigue

  • Dyspnea

  • Signs of right heart failure

PHYSICAL EXAM FINDINGS

  • Right ventricular lift

  • Loud S 1

  • Fixed split S2

  • Opening snap

  • Pulmonary flow murmur

  • Diastolic mitral rumble

DIFFERENTIAL DIAGNOSIS

  • Mitral stenosis with the opening snap mistaken for a wide fixed split S2 and another systolic murmur (eg, innocent flow, mild mitral, or tricuspid regurgitation) mistaken for a pulmonary outflow murmur

  • ASD with the wide fixed split S2 mistaken for an opening snap and a tricuspid flow rumble mistaken for the murmur of mitral stenosis

  • Ebstein’s anomaly of the tricuspid valve and an ASD

DIAGNOSTIC EVALUATION

ELECTROCARDIOGRAPHY

  • Right ventricular hypertrophy

  • Right and left atrial abnormality

  • Atrial fibrillation

IMAGING STUDIES

  • Echocardiography: demonstrates the thickened stenosed mitral valve and the ASD

  • Doppler echocardiography: can quantitate the mitral valve area, the shunt fraction across the ASD, and the severity of any pulmonary hypertension

  • Cardiac MRI is useful for quantitating the shunt flow and the severity of mitral stenosis

DIAGNOSTIC PROCEDURES

  • Transesophageal echocardiography: may be needed to define the atrial septal anatomy and the location of the ASD

  • Cardiac catheterization: may be necessary to confirm the severity of mitral stenosis, the magnitude of the shunt, and the severity of any pulmonary hypertension

TREATMENT

CARDIOLOGY REFERRAL

  • Symptoms with signs of valvular or congenital heart disease

  • Atrial fibrillation

  • Heart failure

  • Paradoxical embolism

  • Suspected endocarditis

HOSPITALIZATION CRITERIA

  • Heart failure

  • Atrial fibrillation with rapid ventricular response

  • Stroke

  • Suspected endocarditis

MEDICATIONS

  • Rate control of atrial fibrillation

  • Anticoagulation with warfarin

THERAPEUTIC PROCEDURES

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