Pulmonary Pseudofibrosis in Cyanotic Heart Disease: A Clinical Syndrome Mimicking Tuberculosis in Patients with Extreme Pulmonic Stenosis

Abstract

Fifteen cyanotic patients with extreme pulmonic stenosis showed radiologic changes of apical pleural capping and parenchymal pseudofibrosis simulating chronic granulomatous chest disease. Repeated skin tests and sputum examination were negative except for one patient who developed terminal opportunistic infection with Aspergillus. The changes are ascribed to two major vascular complications of extreme pulmonic stenosis: (1) apical capping caused primarily by a plexus of transpleural collateral vessels and pleuritis following multiple small bleeds from these vessels; (2) parenchymal lesions caused both by varicosities of transpleural, bronchial and other collateral vessels and by thromboses in small pulmonary arteries. The syndrome occurred in patients with persistent cyanosis and polycythemia due to pulmonic stenosis who survived for 15 or more years. In some, apical pseudofibrotic changes were accompanied by recurrent small hemoptyses. If results of appropriate skin testing and sputum examination prove negative for granulomatous disease, the diagnosis of “pseudo-” pseudofibrosis of the cyanotic” should be considered in patients with the triad of cyanosis, reduced pulmonary blood flow and chronic apical pulmonary changes. Fifteen cyanotic patients with extreme pulmonic stenosis showed radiologic changes of apical pleural capping and parenchymal pseudofibrosis simulating chronic granulomatous chest disease. Repeated skin tests and sputum examination were negative except for one patient who developed terminal opportunistic infection with Aspergillus. The changes are ascribed to two major vascular complications of extreme pulmonic stenosis: (1) apical capping caused primarily by a plexus of transpleural collateral vessels and pleuritis following multiple small bleeds from these vessels; (2) parenchymal lesions caused both by varicosities of transpleural, bronchial and other collateral vessels and by thromboses in small pulmonary arteries. The syndrome occurred in patients with persistent cyanosis and polycythemia due to pulmonic stenosis who survived for 15 or more years. In some, apical pseudofibrotic changes were accompanied by recurrent small hemoptyses. If results of appropriate skin testing and sputum examination prove negative for granulomatous disease, the diagnosis of “pseudo-” pseudofibrosis of the cyanotic” should be considered in patients with the triad of cyanosis, reduced pulmonary blood flow and chronic apical pulmonary changes.