Radiological Category Thoracic Cardiovascular Principal Modality 1 CT

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Radiological Category: Thoracic Cardiovascular Principal Modality (1): CT Principal Modality (2): General Radiography Case

Radiological Category: Thoracic Cardiovascular Principal Modality (1): CT Principal Modality (2): General Radiography Case Report #0608 Submitted by: Pooja Thakrar, M. D. Faculty reviewer: Emma Ferguson, M. D. Date accepted: 15 April 2009

Case History HPI: 60 yo AAF with h/o hypertension presents with chest pain. Initially

Case History HPI: 60 yo AAF with h/o hypertension presents with chest pain. Initially admitted for seizures and loaded with fosphenytoin; subsequently began to have chest pain. EKG showed atrial fibrillation. PMH: Diabetes, hypertension, prior cerebrovascular accident. PSH: Percutaneous ASD closure in 2006. Medications: Allopurinol, atenolol, hydrocodone, amlodipine, metformin, simvastatin, and Plavix. Social Hx: Denies drug, alcohol, or tobacco use. Family Hx: Unknown. ROS: No additional. PE: Vitals: P 110 BP 96/60 RR 11 Gen: Sedated. Chest: CTA bilaterally. Tachycardic, nl S 1/S 2. No M/R/G. Abdomen: Normoactive BS, soft, NTND. Extremities: 2+ pitting bilateral LEE. T 37

Radiological Presentations

Radiological Presentations

Radiological Presentations

Radiological Presentations

Radiological Presentations

Radiological Presentations

Radiological Presentations

Radiological Presentations

Radiological Presentations

Radiological Presentations

Test Your Diagnosis Which one of the following is your choice for the appropriate

Test Your Diagnosis Which one of the following is your choice for the appropriate diagnosis? After your selection, go to the next page. • Isolated severe tricuspid regurgitation • Ebstein’s anomaly • Levo-transposition of the great arteries • Uhl’s anomaly • Constrictive pericarditis

Findings and Differentials Findings: CXR: Global cardiomegaly CT: Marked cardiac enlargement with massive dilatation

Findings and Differentials Findings: CXR: Global cardiomegaly CT: Marked cardiac enlargement with massive dilatation of right atrium and right ventricle. ASD closure device. Contrast reflux into IVC and hepatic veins reflecting R heart failure. Small pericardial effusion. Differentials: • Ebstein’s anomaly • Isolated severe tricuspid regurgitation • Levo-transposition of the great arteries • Uhl’s anomaly

Discussion Ebstein’s anomaly is a congenital malformation of the right side of the heart

Discussion Ebstein’s anomaly is a congenital malformation of the right side of the heart which results in abnormal anatomy of the tricuspid valve. In normal embryological development, the right ventricular wall is undermined in order to properly form the tricuspid leaflets and chordae. If the undermining is incomplete and does not reach the atrioventricular junction, the result is apical displacement of the posterior and septal leaflets of the valve, i. e. , Ebstein’s anomaly. The anterior leaflet is variably displaced and malformed. These changes result in atrialization of the portion of the right ventricle proximal to the valve, meaning that the normal structure of the ventricular wall is lost and it instead resembles that of the right atrium. Given the origin of the tissue, however, the part of the ventricular wall in the atrial chamber continues to contract with the remainder of the ventricle. This, in combination with a variable degree of tricuspid regurgitation, results in backflow of blood from the right ventricle to the right atrium and, if severe enough, eventual right-sided heart failure. The etiology of Ebstein’s anomaly is often uncertain, but the classic association is of maternal lithium use during the first trimester of pregnancy. The average of diagnosis is in the mid-teenage years, and only about five percent of patients survive past age 50. Those survivors tend to be patients with less severe malformations. Associated anomalies include atrial septal defects and patent foramen ovale.

Discussion Apically displaced tricuspid valve Atrialized right ventricular wall In our patient, the right

Discussion Apically displaced tricuspid valve Atrialized right ventricular wall In our patient, the right atrium appears grossly enlarged. However, it is important to recognize that the chamber which appears to represent the right atrium in fact contains both right atrial and right ventricular tissue, as the tricuspid valve is apically displaced. ASD closure device Interventricular septum bowed into left ventricle The patient had a prior atrial septal defect repair. However, the interventricular septum is bowed into the left ventricle, indicating right heart pressures above those in the left side of the heart. This is a result of longstanding left-to-right shunting through the ASD prior to the repair, causing right-sided hypertrophy and eventual reversal of the shunt.

Discussion Contrast reflux into IVC and hepatic veins Her atrial fibrillation is a result

Discussion Contrast reflux into IVC and hepatic veins Her atrial fibrillation is a result of right atrial enlargement, and her lower extremity edema is a sequela of right-sided heart failure. Reflux of contrast into the inferior vena cava and hepatic veins is a result of this right-heart failure and venous congestion.

Discussion Isolated, severe tricuspid regurgitation can certainly result in heart failure, as seen in

Discussion Isolated, severe tricuspid regurgitation can certainly result in heart failure, as seen in this patient, but the tricuspid valve would not be malpositioned. In our patient, the tricuspid valve is apically displaced, resulting in atrialization of the portion of the right ventricle which remains proximal to the valve. The right atrium is enlarged as a result of triscuspid regurgitation, but the apparent size of the right atrium is even larger, given the additional atrialized right ventricular tissue. Therefore, while tricuspid regurgitation plays a role in the patient’s pathology, it does not represent the underlying malformation. Levo-transposition of the great arteries is sometimes referred to as “congenitally corrected” transposition of the great arteries because the anomaly involves both atrioventricular discordance and ventriculoarterial discordance. What this means is that the right atrium empties into the left ventricle, which then empties into the pulmonary artery. Similarly, the left atrium empties into the right ventricle, which then sends blood into the aorta. These distinctions are made based on the anatomy of the respective ventricles (e. g. , coarse trabeculations and a tricuspid valve in the right ventricle versus fine trabeculations and a mitral valve in the left ventricle). Though the ventricles are “switched, ” the pulmonary and systemic circulations are preserved. In our patient, no atrioventricular or ventriculoarterial discordance is seen.

Discussion Uhl’s anomaly is a very rare disorder characterized by congenital complete or partial

Discussion Uhl’s anomaly is a very rare disorder characterized by congenital complete or partial absence of the right ventricular myocardium, resulting in apposition of the epicardium and endocardium. The right ventricle is therefore unable to contract, leading to backup of blood in the right atrium and right-sided heart failure. In a patient with Uhl’s anomaly, unlike in our patient, the tricuspid valve would maintain its normal position.

Discussion The gold standard for diagnosis of Ebstein’s anomaly is echocardiography. Our patient had

Discussion The gold standard for diagnosis of Ebstein’s anomaly is echocardiography. Our patient had an echocardiogram which demonstrated a small left ventricle “imprisoned” by a severely dilated but functional right atrium with resultant impairment of left ventricular systolic function. The septal motion is paradoxic due to right ventricular pressure overload. The right ventricle is almost completely obliterated as a result of apical displacement of the tricuspid valve, and the tricuspid valve is abnormal with a hypoplastic septal leaflet and a large anterior leaflet. Severe tricuspid regurgitation is apparent, and reversal of hepatic flow is seen.

Diagnosis Ebstein’s anomaly

Diagnosis Ebstein’s anomaly

References 1. Riaz K, et al. Ebstein Anomaly. Emedicine 2008. 2. Brickner M, et

References 1. Riaz K, et al. Ebstein Anomaly. Emedicine 2008. 2. Brickner M, et al. Congenital Heart Disease in Adults. The New England Journal of Medicine 2000; 342. 3. Gessner I. Ventricular Inversion. Emedicine 2007. 4. Gerlis L. Uhl’s Anomaly. Orphanet 2003. 5. Attenhofer Jost C, et al. Ebstein’s Anomaly. Circulation 2007; 115: 277 -285.