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Pulmonary Capillary Hemangiomatosis (PCH) in a Patient With Cardiac Amyloidosis: Is There a Link?
Obstructive Lung Diseases SESSION TITLE: Fellow Case Report Poster - Obstructive Lung Disease SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM
Pulmonary Capillary Hemangiomatosis (PCH) in a Patient With Cardiac Amyloidosis: Is There a Link? Hafiz Abdul Moiz Fakih MD* Salim Daouk MD Susheela Hadigal MD; and Michael Jantz MD University of FloridaGainesville, Gainesville, FL INTRODUCTION: Pulmonary capillary hemangiomatosis (PCH) is a rare condition, characterized by proliferation of capillaries that involve alveolar walls, vessels and airways. Given the exact etiology and nature of PCH is unknown, various theories and associations have been proposed. We present a case of PCH, in patient with infiltrative cardiomyopathy, found to be cardiac amyloidosis and pulmonary hypertension.
DISCUSSION: Some authors have suggested that PCH may be neoplastic, on the basis of cellular atypia and invasiveness, but capillary alterations stimulating PCH have been reported to be associated with the severe form of pulmonary venous congestion (SPVC)(1). Our review of literature reported only one case that had PCH associated with Idiopathic Hypertrophic cardiomyopathy. To our knowledge this is the only case with cardiac amyloidosis and PCH. CONCLUSIONS: The challenge in this unique case is whether PCH is a resultant of the cardiac amyloidosis or these are two separate entities. Accumulating cases, including the present one, indicate a causal relationship between PCH and SPVC, although the exact pathogenesis of PCH is still unclear. Reference #1: 1. Matsukuma S, Sato K. Pulmonary capillary haemangiomatosis like lesions in severely congested lungs. Histopathology. 2011;59(5):876-81. DISCLOSURE: The following authors have nothing to disclose: Hafiz Abdul Moiz Fakih, Salim Daouk, Susheela Hadigal, Michael Jantz No Product/Research Disclosure Information DOI:
http://dx.doi.org/10.1016/j.chest.2016.08.1003
Copyright ª 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.
journal.publications.chestnet.org
903A
OBSTRUCTIVE LUNG DISEASES
CASE PRESENTATION: A 63-year-old male, was referred to our clinic for evaluation of bilateral lung nodules. He has a 10pack-year smoking history, quit 25 years back, who had a CT chest for evaluation of cough that had started an year ago. It was associated with weight loss, hemoptysis and exertional dyspnea. He has hypertension, treated with olmesartan, currently taken off due to orthostasis. His physical exam, including vital signs, and labs (CBC, CMP, PFTs) were within normal limits. His CT chest showed bilateral multiple ground glass nodules and mediastinal adenopathy. He underwent an EBUS-TBNA, which was negative for malignancy, showing benign lymphoid tissue. After a scan in 3 month demonstrated interval worsening in number of these nodules, he underwent a VATS wedge biopsy, which was consistent with PCH, confirmed by two pathology referral centers. His echo showed an EF of 65%, severe biventricular hypertrophy, severe diastolic dysfunction (LA size 5.2 cm) and an RVSP of 45 mm Hg. He underwent a right heart cath: RA mean 13 mmHg, RV 60/11, PA 65/20, PA mean 36, PCWP 22, PVR 3.02 woods unit, CI 2.19 and CO (thermodilution) 4.63 L/min. His endomyocardial biopsy demonstrated cardiac amyloidosis.
Pulmonary Capillary Hemangiomatosis (PCH) in a Patient With Cardiac Amyloidosis: Is There a Link? Hafiz Abdul Moiz Fakih MD* Salim Daouk MD Susheela Hadigal MD; and Michael Jantz MD University of FloridaGainesville, Gainesville, FL INTRODUCTION: Pulmonary capillary hemangiomatosis (PCH) is a rare condition, characterized by proliferation of capillaries that involve alveolar walls, vessels and airways. Given the exact etiology and nature of PCH is unknown, various theories and associations have been proposed. We present a case of PCH, in patient with infiltrative cardiomyopathy, found to be cardiac amyloidosis and pulmonary hypertension.
DISCUSSION: Some authors have suggested that PCH may be neoplastic, on the basis of cellular atypia and invasiveness, but capillary alterations stimulating PCH have been reported to be associated with the severe form of pulmonary venous congestion (SPVC)(1). Our review of literature reported only one case that had PCH associated with Idiopathic Hypertrophic cardiomyopathy. To our knowledge this is the only case with cardiac amyloidosis and PCH. CONCLUSIONS: The challenge in this unique case is whether PCH is a resultant of the cardiac amyloidosis or these are two separate entities. Accumulating cases, including the present one, indicate a causal relationship between PCH and SPVC, although the exact pathogenesis of PCH is still unclear. Reference #1: 1. Matsukuma S, Sato K. Pulmonary capillary haemangiomatosis like lesions in severely congested lungs. Histopathology. 2011;59(5):876-81. DISCLOSURE: The following authors have nothing to disclose: Hafiz Abdul Moiz Fakih, Salim Daouk, Susheela Hadigal, Michael Jantz No Product/Research Disclosure Information DOI:
http://dx.doi.org/10.1016/j.chest.2016.08.1003
Copyright ª 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.
journal.publications.chestnet.org
903A
OBSTRUCTIVE LUNG DISEASES
CASE PRESENTATION: A 63-year-old male, was referred to our clinic for evaluation of bilateral lung nodules. He has a 10pack-year smoking history, quit 25 years back, who had a CT chest for evaluation of cough that had started an year ago. It was associated with weight loss, hemoptysis and exertional dyspnea. He has hypertension, treated with olmesartan, currently taken off due to orthostasis. His physical exam, including vital signs, and labs (CBC, CMP, PFTs) were within normal limits. His CT chest showed bilateral multiple ground glass nodules and mediastinal adenopathy. He underwent an EBUS-TBNA, which was negative for malignancy, showing benign lymphoid tissue. After a scan in 3 month demonstrated interval worsening in number of these nodules, he underwent a VATS wedge biopsy, which was consistent with PCH, confirmed by two pathology referral centers. His echo showed an EF of 65%, severe biventricular hypertrophy, severe diastolic dysfunction (LA size 5.2 cm) and an RVSP of 45 mm Hg. He underwent a right heart cath: RA mean 13 mmHg, RV 60/11, PA 65/20, PA mean 36, PCWP 22, PVR 3.02 woods unit, CI 2.19 and CO (thermodilution) 4.63 L/min. His endomyocardial biopsy demonstrated cardiac amyloidosis.