Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content +++ 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 ... Your Access profile is currently affiliated with '[InstitutionA]' and is in the process of switching affiliations to '[InstitutionB]'. Please click ‘Continue’ to continue the affiliation switch, otherwise click ‘Cancel’ to cancel signing in. Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Password? Forgot Username? Sign in via OpenAthens Sign in via Shibboleth You already have access! Please proceed to your institution's subscription. Create a free a profile for additional features.