Cardionerds: A Cardiology Podcast

345. Case Report: A Case of Unrepaired Congenital Heart Disease – University of Chicago – Northshore University

11.22.2023 - By CardioNerdsPlay

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CardioNerds (Dr. Josh Saef, Dr. Agnes Koczo) join Dr. Iva Minga, Dr. Kifah Hussain, and Dr. Kevin Lee from the University of Chicago - NorthShore to discuss a case of unrepaired congenital heart disease that involves D-TGA complicated by Eisenmenger syndrome. The ECPR was provided by Dr. Michael Earing. Audio editing by Dr. Akiva Rosenzveig.

A 25-year-old woman with an unknown congenital heart disease that was diagnosed in infancy in Pakistan presents to the hospital for abdominal pain and weakness. She is found to be profoundly hypoxemic, and an echocardiogram revealed D-transposition of the great arteries (D-TGA) with a large VSD. As this was not repaired in childhood, she has unfortunately developed Eisenmenger syndrome with elevated pulmonary vascular resistance. She is stabilized and treated medically for her cyanotic heart disease. Unfortunately given the severity and late presentation of her disease, she has limited long-term options for care. CardioNerds discuss the diagnosis of D-TGA and Eisenmenger’s syndrome, as well as long-term management and complications associated with this entity.

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Case Media - Unrepaired Congenital Heart Disease

Pearls - Unrepaired Congenital Heart Disease

* Early diagnosis of cyanotic congenital heart disease is paramount for treatment and prevention of future complications.

* Adult congenital heart disease requires a multi-disciplinary team for management in consultation with an adult congenital cardiology specialist.

* Eisenmenger syndrome is related to multiple systemic complications and has a high rate of mortality.

* Advancement in PAH medical management can offer noninvasive treatment options for some patients.

* Transthoracic echocardiography is the cornerstone for diagnosis.

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