Uncorrected Univentricular Heart in an Adult

Key words: congenital heart defect, coronary imaging

An 18-year-old male patient with an unknown past medical history presented to the emergency room with complaints of palpitations and paroxysmal episodes of shortness of breath over a 7-year period. The patient was from Uruguay and visiting the United States when symptoms occurred. He had been told in the past that he had a heart defect, but was never treated surgically or medically. On physical examination, the patient was slightly tachycardic with a systolic murmur noted adjacent on the left fourth intercostal space. Crackles were noted in the lower lung fields bilaterally. The patient looked adequately nourished with average build, along with grade 1 clubbing of his digits. Blood pressure was 100/64 mm Hg with a heart rate around 120 beats/min, saturating around 95% on room air. His dyspnea improved with supplemental oxygen over several minutes. Electrocardiogram demonstrated atrial flutter. Chest x-ray showed moderate pleural effusions bilaterally with a smaller-than-expected heart. Transthoracic echocardiography displayed a univentricle with an ejection fraction of approximately 45% (Figure 1 and Video 1). No other cardiac pathologies were noted. Cardiac catheterization and surgical interventional options were presented to the patient, but he refused and was lost to follow-up.

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From ¹the Department of Anesthesia, Mount Sinai Medical Center, Miami Beach, Florida; and the ²Echocardiography Laboratory, Columbia University Division of Cardiology at the Mount Sinai Heart Institute, Miami Beach, Florida.

Disclosure: The authors have completed and returned the ICMJE Form for Disclosure of Potential Conflicts of Interest. The authors report no conflicts of interest regarding the content herein.

The authors report that patient consent was provided for publication of the images used herein.

Manuscript accepted April 15, 2019.

Address for correspondence: Orlando Santana, MD, Director, Echocardiography Laboratory, Columbia University Division of Cardiology, Mount Sinai Heart Institute, 4300 Alton Road, Miami Beach, FL 33140. Email: osantana@msmc.com