A Holey Predicament

Abstract

SESSION TITLE: Student/Resident Case Report Poster - Cardiovascular Disease II SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Endocardial cushion defects are congenital abnormalities that result in valvular dysfunction as well as “holes” in the septa of the heart. With medical advances, these abnormalities are typically diagnosed very early in life. Although survival into adulthood is possible, these patients develop a multitude of complications that can be life threatening. CASE PRESENTATION: A seventy-two year-old female with recently diagnosed atrial fibrillation presented to the emergency room with dyspnea and palpitations. On physical exam, she had an irregular tachycardia and a systolic murmur. Chest radiography demonstrated severe cardiomegaly. An electrocardiogram showed an atrial tachyarrhythmia, possibly multifocal atrial tachycardia. An infusion of diltiazem was started. She converted to sinus rhythm and symptoms improved. A few hours later, she suffered a sudden cardiopulmonary arrest (CPA). CPR was initiated during which intubation was performed. She had return of spontaneous circulation after fifteen minutes but remained severely hypoxic despite multiple ventilator changes and administration of a paralytic agent. However, oxygenation improved with inhaled nitric oxide therapy. An echocardiogram performed prior to the CPA provided some answers; there was a septum primum atrial septal defect measuring 2.3cm with severe pulmonary hypertension and Eisenmengers syndrome. Interestingly, the initially observed right-to-left shunt reversed with the delivery of nitric oxide. A transesophageal echocardiogram showed an aneurysm of the perimembranous portion of the ventricular septum where a ventricular septal defect may have been previously present. A single atrioventricular valve annulus with two separate orifices was present, thus categorizing the endocardial cushion defect as either partial or transitional. A right heart catheterization demonstrated pulmonary artery pressure of 61/6mmHg, pulmonary artery wedge pressure of 13mmHg and ratio of pulmonary to systemic blood flow (Qp/Qs) of three. The patient was not a surgical candidate because of the presence of Eisenmenger syndrome. She recovered with supportive critical care, was extubated and discharged home in stable condition. DISCUSSION: Eisenmenger syndrome is a complication of pulmonary hypertension associated with congenital heart disease whereby increased pulmonary blood flow leads to pulmonary vascular resistance and shunt reversal. These patients are at high risk for developing atrial arrhythmias, heart failure and sudden cardiac death. Surgical correction of the abnormalities is usually performed at an age less than six months and is not recommended after the development of shunt reversal. CONCLUSIONS: This case highlights the diagnostic workup, potential dangerous consequences as well as critical care management of endocardial cushion defects in an adult. Reference #1: Vongpatanasin W, Brickner ME, Hilis LD and Lange RA. The Eisenmenger syndrome in adults. Ann Intern Med. 1998;128(9):745-55. DISCLOSURE: The following authors have nothing to disclose: Shaiva Meka, Daniel Sheldon, Paul Christensen No Product/Research Disclosure Information