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A3722 - Unfolding a Rare Case of Frank Hemoptysis: In History Lies All the Secrets!
Author Block: A. Z. Rasheed1, M. Shahzadi2, L. N. Gerolemou3, V. P. Vasudevan4, F. Arjomand5; 1Pulmonary Medicine, The Brooklyn Hospital Center, Brooklyn, NY, United States, 2Pulmonology, Brooklyn, NY, United States, 3The Brooklyn Hospital Center, Brooklyn, NY, United States, 4Pulmonary Division, Internal Medicine, The Brooklyn Hospital, Brooklyn, NY, United States, 5Brooklyn Hosp Ctr, Brooklyn, NY, United States.
Introduction: The Fontan procedure was first used in 1971 for the surgical management of tricuspid atresia, and it has become the definitive palliative surgical treatment for all forms of univentricular congenital heart disease. Hemoptysis is an extremely rare but potentially fatal complication attributed to the bleeding from aortopulmonary collateral vessels. We describe a patient with frank hemoptysis after 15 years of undergoing Fontan procedure and we report a successful treatment with coil embolization.Case presentation: 26 years old gentleman with no history of bleeding disorder presented with sudden onset of hemoptysis. He was not on any anti platelet or anticoagulation therapy. He had history of hypoplastic right heart syndrome with tricuspid and pulmonary valve atresia. He had underwent bilateral Blalock-Taussing shunts in infancy, Fontan operation at age of 8 and mitral valve repair for severe mitral prolapse 3 years ago. He had no history of substance abuse and had 1 pack year's history of smoking. He had grade II/VI holosytolic murmur at apex. Lungs were clear and had no remarkable findings on skin examination. He had no leukocytosis and had normal platelet counts. His liver function test, renal function and coagulation profile was normal. Xray chest was normal. CT chest revealed patchy opacities in the right upper lobe. Echocardiogram showed no right ventricle, normal contractility of single ventricle and mild mitral regurgitation. Cardiac cath showed good pressure throughout Fontan pathway SVC=LPA=RPA=Fontan=8mmHg. RPCWP was 3mmHg and LPCW was 4mmHg. There were multiple aortopulmonary collateral, mostly small ones. One large one draining from proximal descending aorta bilaterally to pulmonary vascular bed was coil occluded. His hemoptysis resolved and he was discharged home. Discussion: Incidence of hemoptysis as long-term complication following Fontan is not well known due to rarity of the disease. Precise causes of aortopulmonary collateral vessels following the Fontan procedure are not known. A decreased volume, velocity and pulsatility of flow in the pulmonary arteries have been suggested as potential stimuli. A history of one or more Blalock-Taussig shunt procedures has been associated with the presence of collateral vessels. In a case series of two cases; there was a persistent right-to-left atrial shunt, with oxygen saturation lower than 91% in both cases. Our patient has no evidence of hypoxemia. Coil embolization of aortopulmonary collateral vessels seems effective treatment.