Abstract 14316: Blue Again: Recurrent Cyanosis in a 30-Year-Old Man with Surgically Palliated Cyanotic Congenital Heart Disease
Background: Patients with complex congenital heart disease are increasingly surviving to adulthood. Case: A 30-year-old man presented with dyspnea and blue lips. He was diagnosed at birth with tricuspid atresia. He underwent surgeries for right-sided Glenn, atriopulmonary Fontan later revised with lateral tunnel, left-sided Glenn, biatrial MAZE and epicardial pacing. At age 26, his left ventricular (LV) function was normal, oxygen saturation 92% on room air, and exercise tolerance unlimited; he was anticoagulated for a laminar thrombus in the lateral tunnel. Transthoracic echocardiography (TTE) at age 29 showed persistent thrombus and moderately depressed LV function. He felt well until 2 weeks earlier, when he developed flu-like symptoms. Symptoms resolved over one week, but he became dyspneic at rest with blue lips. He was afebrile, with a regular pulse of 82 bpm, blood pressure 112/80 mmHg, and 24 breaths per minute. Oxygen saturation was 81% with and without oxygen support. Examination revealed elevated weight, central cyanosis, clear lungs, right upper quadrant tenderness, lukewarm extremities without edema, and nail clubbing and cyanosis with delayed capillary refill. Rhythm was sinus, and pacemaker interrogation showed no recent tachyarrhythmia. TTE showed growth of known thrombus and severe LV dysfunction. Differential diagnosis included Fontan circuit obstruction, pulmonary thromboembolism, and arteriovenous or venous collateral shunting. Left atrial hypertension from LV failure could further diminish Fontan flow. Transesophageal echocardiography showed massive clot progression, nearly obliterating the lateral tunnel. Strategy was redirected to thrombolysis and possible transplantation. Computed tomography was arranged to assess clot burden, but during scanning, the patient arrested, with failure to restore spontaneous circulation.
Conclusions: This case highlights the perils associated with recurrent cyanosis in a patient with surgically palliated congenital cyanotic heart disease. Concurrent LV failure confers a poor prognosis, and recognition should prompt close monitoring and early referral for transplant evaluation, as LV failure precludes isolated Fontan revision.
- © 2012 by American Heart Association, Inc.