INTRODUCTION: Bronchial variceal bleeding is a rare occurrence and has been reported in mitral stenosis and portal hypertension. We present a case of hemoptysis in a patient with bronchial varices secondary to failing Fontan circulation 29 years post-operatively.
CASE PRESENTATION: A 45 year old male admitted to the hospital after a right heart catheterization for hemoptysis. The patient had a total of three episodes. The quantity of blood with each episode decreased and was described as maroon, thick, semi-clotted, and totaled roughly 200mL of blood. He had a previous episode two weeks prior this current presentation. He smoked one pack a day for the past 30 years, but quit three months prior to presentation. He has no exposure to tuberculosis nor is there any family history of lung malignancy. The patient was being evaluated for heart failure and possible transplantation in the setting of failing Fontan circulation. The patient was born with tricuspid atresia and underwent a Potts Shunt (descending aorta to left pulmonary artery anastomosis) at birth. Subsequently, he required a Fontan operation with a right atrial appendage to pulmonary artery connection in 1981. Other past medical history included stage II chronic kidney disease and obstructive sleep apnea. Medications included bumetanide, aspirin, and metolazone. Physical examination was remarkable for cyanotic fingers and pitting edema in the lower extremities. His right heart catheterization revealed a mean right atrial pressure of 21mmHg, mean right pulmonary artery pressure of 20mmHg, mean left pulmonary artery pressure of 21mmHg, pulmonary capillary wedge pressure of 20mmHg, and a cardiac index of 2.4 L/min/m2. Laboratory evaluation was unremarkable. The patient underwent fiberoptic bronchoscopy that revealed hyperemia and hypervascularity of the larynx and trachea. Additionally, the right and left mainstem bronchi demonstrated hypervascularity and varicosities that extended into the lower lobes bilaterally. Given the extent of the varices and lack of active bleeding, no intervention was undertaken. Sildenafil was added to the patient’s medication regimen and transplantation evaluation continued. Four months later the patient underwent evaluation for ascites of 6 weeks duration. A liver biopsy was performed and revealed histologic features consistent with venous outflow obstruction and early cirrhosis due to passive congestion. He has not had any further episodes of hemoptysis and continues with transplantation evaluation.
DISCUSSION: Bronchial varices are rare and have been previously reported in mitral stenosis and portal hypertension. Hemoptysis in patients with Fontan physiology secondary to aortopulmonary collateral vessels has also been reported. Primary circulation after Fontan surgery results in a continuous column of blood from the aorta to the systemic capillaries and veins as well as the pulmonary capillaries and veins. This leads to non-pulsatile pulmonary blood flow and 70% of the total blood volume in venous circulation. Collateral circulation can also develop in the bronchial vessels. As the Fontan fails, the pressure from this volume of blood is transferred into the bronchial circulation. This results in passive congestion and engorgement. Our patient had extensive varicosities throughout his trachea and bronchi with visual evidence of engorgement. The risk of exsanguination is high given the elevated pressure in a system accustomed to lower levels of pressure. Treatment is targeted at decreasing the overall pressure in circulation and management of the failing single ventricle.
CONCLUSIONS: Bronchial varices represent a rare cause of hemoptysis. As patients’ with congential heart disease continue to live longer, bronchial varices may become more prevalent and will require serious consideration by clinicians given the risk of exsanguination.
Reference #1 De Leval, MR & Deanfield, JE. Four decades of fontan palliation. Nature Reviews Cardiology. 2010; 7: 520-527.
Reference #2 Suda, K, Matsumura, M, & Sano, A, et. al. Hemoptysis from collateral arteries 12 years after a fontan-type operation. Annals of Thoracic Surgery. 2005; 79: e7-8.
Reference #3 Youssef, YR, Escalante-Glorsky, S, & Bonnet, RB, et al. Hemoptysis secondary to bronchial varicies associated with alchoholic liver cirrhosis and portal hypertension. The American Journal of Gastroenterology. 1994; 89: 1562-1563
DISCLOSURE: The following authors have nothing to disclose: Richard Patch, Jennifer Mattingley, Eric Edell, Ulrich Specks
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