Pulmonary Vascular Disease: Thoracic Vascular Disorders |

Extrinsic Compression of Pulmonary Vein and Left Atrium by Gigantic Pulmonary Artery Aneurysm: A Unique Presentation of Tuberous Sclerosis FREE TO VIEW

Kaushal Patel, MD; Marvin Balaan, MD
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Allegheny General Hospital, Pittsburgh, PA

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):1230A. doi:10.1016/j.chest.2016.08.1339
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SESSION TITLE: Thoracic Vascular Disorders

SESSION TYPE: Student/Resident Case Report Slide

PRESENTED ON: Monday, October 24, 2016 at 11:00 AM - 12:00 PM

INTRODUCTION: Tuberous sclerosis complex (TSC) is an autosomal dominant condition that is characterized by hamartomas of viscera and haemangiomas of small blood vessels. Aneurysms of medium/large vessels are rare and have been reported with aorta; renal, brachial, axillary and intracranial arteries. Pulmonary artery (PA) aneurysm itself is very rare on its own. Common etiologies include infection, cardiac and vascular abnormalities including Bechet’s, pulmonary hypertension, and trauma. Less than 5 cases of primary PA aneurysm (PAA) related to TSC have been reported. We are reporting an interesting and to our knowledge, the first reported case of pulmonary vein(PV)/Left atrial(LA) compression by gigantic PAAs associated with TSC.

CASE PRESENTATION: 67-year-old white female with past history of TCS, type V pulmonary hypertension due to lymphangioleiomyomatosis, presented to the emergency room with worsening dyspnea, orthopnea. Initial CT scan showed pulmonary edema and enlarged PAs with multiple aneurysm formations. Common causes of PAA were ruled out based on history and laboratory findings. Although smooth muscle cell proliferation was not histologically proven, the presence of several fusiform aneurysms with segmental arterial wall thickening supports the hypothesis of diffuse vascular wall abnormalities related to the dysplastic nature of TSC. She required noninvasive ventilation with very high FiO2 even after diuretics. Her pulmonary edema appeared mostly right-sided. With suspicion of LA compression, CT scan with LA protocol was performed. It showed compression of LA and right superior PV due to aneurismal PA. She underwent PV stent placement through trans-septal approach with dramatic improvement in her symptoms, imaging and O2 requirement.

DISCUSSION: TSC is characterised by the presence of multiorgan hamartomas related to TSC1 or TSC2 gene mutations. Hamartomas in TSC are more frequent in the skin, brain, kidneys and can occur in the lung, heart, small blood vessels, but very seldom in large and medium sized vessels. PAA with TCS is exceptionally rare and has only been reported in 5 cases. All except one case were solitary aneurysms. We report here a unique case of TSC presenting with gigantic PA (main PA 7.8cm, left PA- 6.2cm) along with multiple PAAs, compressing right PV/RA and presented as respiratory failure. To our knowledge, there has not been any reported case of PV/LA compression caused by PAA.

CONCLUSIONS: Vascular wall abnormalities due to the dysplastic nature of TSC may rarely affect PA and can lead to PAAs as mentioned in our case. It is important to recognize them early on as their proximity to important structures and tendency to rupture can cause serious complications. A high index of suspicion is required to diagnose it.

Reference #1: Burrows NJ, Johnson SR. Pulmonary artery aneurysm and tuberous sclerosis. Thorax. 2004;59(1):86

Reference #2: Dunet V, Qanadli SD. Multiple pulmonary artery aneurysms in tuberous sclerosis complex. BMJ Case Rep. 2013;2013

DISCLOSURE: The following authors have nothing to disclose: Kaushal Patel, Marvin Balaan

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