35 Ebstein’s anomaly Instruction Listen to this patient’s heart. He was told he had an innocent murmur during a school medical examination many years ago but now has a large globular heart on chest radiography. Salient features History • An incidental cardiac murmur • Ask the patient about palpitations (paroxysmal supraventricular tachycardia) • Symptoms of right-sided heart failure • History of maternal lithium ingestion. Examination • Raised jugular venous pulse; the large ‘v’ of tricuspid regurgitation is absent because the giant right atrium absorbs most of the regurgitant volume • Left parasternal heave • Loud first heart sound produced by the sail-like anterior tricuspid leaflet • Pansystolic murmur which increases on inspiration • Hepatomegaly • Proceed to: • ascertain whether the patient has exertional cyanosis or dyspnoea • exclude an atrial septal defect. Diagnosis This patient has isolated tricuspid regurgitation (lesion) that is probably of congenital aetiology as there is no pulmonary hypertension. He has Ebstein’s anomaly with cardiomegaly and cardiac failure (functional status). Advanced-level questions What is the pathology in Ebstein’s anomaly? The tricuspid leaflets are abnormal and are displaced into the body of the RV. The septal leaflet is variably deficient or even absent. The posterior leaflet is also variably deficient and there is large ‘sail-like’ anterior leaflet, which is the hallmark of this condition. The anterior leaflet is rarely affected. The abnormally located tricuspid orifice produces a part of the RV lying between the atrioventricular ring and the origin of the valve, which is continuous with the right atrial chamber. This proximal segment is known as the ‘atrialized’ portion of the RV. Approximately 50% of the patients have either a patent foramen ovale or a secundum atrial septal defect, and 25% have one or more accessory atrioventricular conduction pathways. The anomaly is said to be associated with maternal lithium ingestion. Only gold members can continue reading. Log In or Register to continue Share this:Click to share on Twitter (Opens in new window)Click to share on Facebook (Opens in new window)Like this:Like Loading... Related Related posts: Permanent cardiac pacemaker/implantable cardioverter-defibrillator Cauda equina syndrome Dermatomyositis Dystrophia myotonica Stay updated, free articles. Join our Telegram channel Join Tags: 250 Cases in Clinical Medicine Dec 4, 2016 | Posted by admin in GENERAL & FAMILY MEDICINE | Comments Off on Ebstein’s anomaly Full access? Get Clinical Tree
35 Ebstein’s anomaly Instruction Listen to this patient’s heart. He was told he had an innocent murmur during a school medical examination many years ago but now has a large globular heart on chest radiography. Salient features History • An incidental cardiac murmur • Ask the patient about palpitations (paroxysmal supraventricular tachycardia) • Symptoms of right-sided heart failure • History of maternal lithium ingestion. Examination • Raised jugular venous pulse; the large ‘v’ of tricuspid regurgitation is absent because the giant right atrium absorbs most of the regurgitant volume • Left parasternal heave • Loud first heart sound produced by the sail-like anterior tricuspid leaflet • Pansystolic murmur which increases on inspiration • Hepatomegaly • Proceed to: • ascertain whether the patient has exertional cyanosis or dyspnoea • exclude an atrial septal defect. Diagnosis This patient has isolated tricuspid regurgitation (lesion) that is probably of congenital aetiology as there is no pulmonary hypertension. He has Ebstein’s anomaly with cardiomegaly and cardiac failure (functional status). Advanced-level questions What is the pathology in Ebstein’s anomaly? The tricuspid leaflets are abnormal and are displaced into the body of the RV. The septal leaflet is variably deficient or even absent. The posterior leaflet is also variably deficient and there is large ‘sail-like’ anterior leaflet, which is the hallmark of this condition. The anterior leaflet is rarely affected. The abnormally located tricuspid orifice produces a part of the RV lying between the atrioventricular ring and the origin of the valve, which is continuous with the right atrial chamber. This proximal segment is known as the ‘atrialized’ portion of the RV. Approximately 50% of the patients have either a patent foramen ovale or a secundum atrial septal defect, and 25% have one or more accessory atrioventricular conduction pathways. The anomaly is said to be associated with maternal lithium ingestion. Only gold members can continue reading. Log In or Register to continue Share this:Click to share on Twitter (Opens in new window)Click to share on Facebook (Opens in new window)Like this:Like Loading... Related Related posts: Permanent cardiac pacemaker/implantable cardioverter-defibrillator Cauda equina syndrome Dermatomyositis Dystrophia myotonica Stay updated, free articles. Join our Telegram channel Join Tags: 250 Cases in Clinical Medicine Dec 4, 2016 | Posted by admin in GENERAL & FAMILY MEDICINE | Comments Off on Ebstein’s anomaly Full access? Get Clinical Tree
