Medical Image of the Month: Diaphragmatic Eventration

Figure 1. An upright PA chest radiograph demonstrates marked elevation of the left hemidiaphragm with associated superior migration of the gas-filled colon and mild mediastinal shift towards the right.

Figure 2. A: frontal. B: sagittal. A non-contrasted reconstruction of the chest demonstrates marked elevation of the left hemidiaphragm with associated superior migration of the abdominal viscera along with preservation of the integrity of the hemidiaphragm. These findings are consistent with a left hemidiaphragm eventration.

Clinical Presentation: A 66-year-old woman presented with a three-year history of progressive postprandial dyspnea and left-sided abdominal pain.  Physical exam revealed normal vital signs and bowels sounds over left lung fields on auscultation. Laboratory work revealed a mild normocytic anemia.  Imaging demonstrated marked left hemidiaphragm elevation with ipsilateral lung parenchyma volume loss and atelectasis along with a mild contralateral mediastinal shift.  A sniff test was consistent with left hemidiaphragm paralysis.

The patient underwent a left video-assisted thoracoscopy, and the left hemidiaphragm was noted to be so thin that the abdominal organs could be visualize through it. The central tendon of the left hemidiaphragm was extremely attenuated and larger than normal. The left hemidiaphragm muscle fibers were noted to be situated around the periphery and not providing any significant tension. The redundant left hemidiaphragm central tendon was excised, and the patient was discharged without symptoms one week later.

Discussion: Eventration of a hemidiaphragm is a rare condition where there is non-paralytic weakening and thinning of a hemidiaphragm resulting in elevation of the hemidiaphragm with retained attachments to the costal margins (1). An eventration usually results from a congenital failure of the fetal diaphragm to muscularized. It is usually unilateral, occurs more on the right than the left, affects the anteromedial portion of the hemidiaphragm, occurs more often in women, and is found after the age of 60 in the adult population. A total eventration of a hemidiaphragm may be indistinguishable from diaphragmatic paralysis and result in a false-positive sniff test – as in this case. When symptomatic, it can pose a diagnostic challenge as it may be confused with a traumatic diaphragmatic rupture in the right clinical setting. Asymptomatic adults do not require treatment.

Leslie Littlefield MD and Mohamed Fayed MD

Department of Pulmonary and Critical Care

University of California San Francisco Fresno

Fresno, CA USA

Reference

1. Black MC, Joubert K, Seese L, et al. Innovative and Contemporary Interventions of Diaphragmatic Disorders. J Thorac Imaging. 2019;34(4):236-247. [CrossRef] [PubMed]

Cite as: Littlefield L, Fayed M. Medical image of the month: diaphragmatic eventration. Southwest J Pulm Crit Care. 2020;21(1):9-10. doi: https://doi.org/10.13175/swjpcc036-20 PDF 

Medical Image of the Month: Double Aortic Arch

Figure 1. A chest radiograph performed after endotracheal intubation of the patient demonstrated prominence of the upper mediastinum in the region of the right paratracheal strip (red arrow).

Figure 2. A non-contrasted CT of the chest demonstrates a complete vascular ring secondary to the patient’s double aortic arch (outlined in red) surrounding the trachea (T) and esophagus (E).

Case Presentation: A 78-year-old gentleman presented to the hospital via EMS with altered mental status. An urgent CT of the head performed in the emergency room demonstrated a large, right intraparenchymal hemorrhage with intraventricular extension into the right lateral ventricle. His Glascow Coma Scale score was 6, and he was intubated for airway protection.  A chest radiograph performed to verify placement of the endotracheal tube demonstrated prominence of the upper mediastinum in the region of the right paratracheal strip (Figure 1). A CT of the chest (Figure 2) demonstrated a double aortic arch corresponding to the upper mediastinal abnormality noted on the chest radiograph. In speaking with the patient’s family after acquiring the CT of the chest, they stated that the patient had long-term issues with dysphagia – specifically choking with solid foods. Unfortunately, the patient passed away from complications of his large intraparenchymal hemorrhage.

A double aortic arch results from persistence of both the right and left embryonic arches with each arch giving rise to the ipsilateral, separate carotid and subclavian arteries (1). A double aortic arch is the most common cause of a symptomatic vascular ring with the trachea and esophagus being compressed by the two arches (1). Symptoms usually arise in childhood with good outcomes with surgical repair of this abnormality in pediatric populations. A few case series exist describing repair of this anomaly in adult populations (2).

Kelly Wickstrom DO, Steven P. Sears DO, and Laura Meinke MD

Division of Pulmonary, Critical Care, Allergy and Sleep Medicine

University of Arizona College of Medicine

Tucson, AZ USA

References

1. Hanneman K, Newman B, Chan F. Congenital variants and anomalies of the aortic arch. Radiographics. 2017 Jan-Feb;37(1):32-51. [CrossRef] [PubMed]
2. Noguchi K, Hori D, Nomura Y, Tanaka H. Double aortic arch in an adult. Interact Cardiovasc Thorac Surg. 2012 Jun;14(6):900-2. [CrossRef] [PubMed]

Cite as: Wickstrom K, Sears SP, Meinke L. Medical image of the month: Double aortic arch. Southwest J Pulm Crit Care. 2019;18(5):120-1. doi: https://doi.org/10.13175/swjpcc019-19 PDF 

Medical Image of the Month: Incarcerated Morgagni Hernia

Figure 1. Lateral view of abdominal-thoracic CT in soft tissue windows.

Figure 2. Coronal view of thoracic CT scan in lung windows.

A Morgagni hernia is a congenital diaphragmatic hernia in which abdominal viscera herniate into the thorax via a defect within an anterior attachment of the diaphragm. As with any bowel-containing hernia, the most feared complication is strangulation with subsequent bowel necrosis. In the present case, a 67-year-old woman presented with a five-day history of acute onset and progressively worsening upper abdominal pain and inability to tolerate oral intake, associated with nausea, vomiting, and mild shortness of breath. A CT revealed a large defect in the right hemidiaphragm consistent with a Morgagni hernia with herniation of the omentum, vessels, and a segment of transverse colon (Figure 1). Findings of bowel ischemia were observed, including (a) pneumatosis intestinalis, seen as cystic foci of air lining the bowel wall, and (b) fluid and fat-stranding adjacent to the affected bowel (Figure 2). Evidence of bowel wall perforation include large volume free air adjacent to the bowel in the right hemithorax and within the abdomen (Figures 1 and 2). Bowel ischemia and necrosis can occur with any hernia and requires prompt diagnosis and management.

Samandip Hothi MD¹ and Viral Patel MD²

¹Department of Medicine, Division of Internal Medicine and ²Department of Medical Imaging

University of Arizona College of Medicine-Tucson

Tucson, AZ USA

References

1. Arora S, Haji A, Ng P. Adult Morgagni Hernia: The Need for Clinical Awareness, Early Diagnosis and Prompt Surgical Intervention. Ann R Coll Surg Engl. 2008 Nov;90(8):694-5. [CrossRef] [PubMed]
2. Ly JQ. The Rigler Sign. Radiology. 2003;228(3):706-7. [CrossRef] [PubMed]
3. Morgan TB, Nguyen DN, Tran CD, Maheshwary RK, Mickus TJ. Morgagni Hernia Causing Incarcerated Bowel and Contributing to Cardiac Arrest. Curr Probl Diagn Radiol. 2018 Jul 31. pii: S0363-0188(18)30181-6. [CrossRef]

Cite as: Hothi S, Patel V. Medical image of the month: Incarcerated Morgagni hernia. Southwest J Pulm Crit Care. 2019;18:59-60. doi: https://doi.org/10.13175/swjpcc001-19 PDF 

Medical Image of the Week: Bronchogenic Cysts

Figure 1. Posterior-anterior (A) and lateral (B) chest radiographs showing a large cyst with an air-fluid level in the right lung.

Figure 2. Representative image from thoracic CT scan in lung windows showing large right lung cyst.

Bronchogenic cysts are congenital foregut malformations forming from abnormal budding of the bronchial tree between the 4^th and 6^th weeks of embryonic development. While identified primarily in children, the cysts are often asymptomatic and may not be identified until adulthood. Most (70%) are within the middle mediastinum and contain fluid or proteinaceous material. When involving the parenchyma, they generally do not communicate with the tracheobronchial tree. Communication with the airways may develop following infection, procedures, or trauma and may result in lesions with an air-fluid level (Figures 1 and 2). Bronchogenic cysts may be complicated by infection, bleeding, fistula formation, or most concerning, by malignant transformation. Unless the cyst contains air, it may manifest as a solitary pulmonary nodule on plain radiographs. Computed tomography or T2-weighted MRI images are used to confirm the diagnosis. 

Steven P. Sears DO¹ and Diana Maria Palacio MD²

¹Division of Pulmonary, Allergy, Critical Care and Sleep and ²Department of Medical Imaging

University of Arizona College of Medicine

Tucson, AZ USA

References

1. McAdams HP, Kirejczyk WM, Rosado-de-Christenson ML, et al. Bronchogenic cyst: Imaging features with clinical and histopathologic correlation. Radiology. 2000 Nov;217(2):441-6. [CrossRef] [PubMed]
2. St-Georges R. Deslauriers J, Duranceau A, et al. Clinical spectrum of bronchogenic cysts of the mediastinum and lung in the adult. Ann Thorac Surg. 1991;52:6-13. [CrossRef] [PubMed]
3. Cardinale L, Ardissone F, Cataldi A, et al. Bronchogenic cysts in the adult: Diagnostic criteria derived from the correct use of standard radiography and computed tomography. Radiol Med. 2008;113(3): 385-94. [CrossRef] [PubMed]

Cite as: Sears SP, Palacio DM. Medical image of the week: Bronchogenic cysts. Southwest J Pulm Crit Care. 2018;16(3):141-2. doi: https://doi.org/10.13175/swjpcc026-18 PDF 

Medical Image of the Week: Pulmonary Artery Sling

Figure 1. Computerized tomography (CT) of the chest showing the aberrant origin of the left pulmonary artery from the right pulmonary artery creating a pulmonary artery sling with mild tracheal narrowing (arrow).

Figure 2. Cardiac magnetic resonance imaging (MRI) confirming the presence of a pulmonary artery sling with aberrant origin of the left pulmonary artery from the right pulmonary artery.

A 42-year-old year woman with asthma was admitted to the hospital with an asthma exacerbation. The patient complained of dyspnea on exertion, two-pillow orthopnea and bipedal edema. An echocardiogram showed a severely dilated right ventricle (RV) with elevated right ventricular systolic pressure of 71 mmHg. The systolic left ventricular (LV) function was also reduced with an ejection fraction of 45%. Computerized tomography (CT) of the chest showed an aberrant origin of the left pulmonary artery (PA) creating a pulmonary artery sling with mild tracheal narrowing (Figure 1, arrow). Cardiac magnetic resonance imaging (MRI) confirmed the presence of a pulmonary artery sling with the aberrant origin of the left PA from the right PA (Figure 2). Cardiac catheterization showed a mean PA pressure of 46mmHg with LV end diastolic pressure of 12mm Hg. The patient was diagnosed with WHO Group I pulmonary hypertension and started on treatment with sildenafil with a stable outpatient course.

Pulmonary artery sling is an uncommon form of vascular ring. The anomaly is a result of formation of the left PA from the right sixth vascular arch (rather than the left), leading to the left PA arising from the posterior aspect of the right PA (1). Pulmonary artery slings may produce symptoms of airway compression and esophageal compression and usually presents in childhood (2). In asymptomatic cases, a PA sling may mimic a mediastinal mass on chest radiographs and CT and MRI may be used to establish the diagnosis (3).

Abhinav Agrawal MD¹, Stuart L Cohen MD², Rakesh Shah MD², Arunabh Talwar MD FCCP¹

¹Division of Pulmonary, Critical Care & Sleep Medicine, Department of Medicine

²Division of Thoracic Radiology, Department of Radiology

Hofstra-Northwell School of Medicine

New Hyde Park, NY USA

References

1. Casta-er E, Gallardo X, Rimola J, Pallardó Y, Mata JM, Perendreu J, Martin C, Gil D. Congenital and acquired pulmonary artery anomalies in the adult: radiologic overview. Radiographics. 2006 Mar-Apr;26(2):349-71. [CrossRef] [PubMed]
2. Odell DD, Gangadharan SP, Majid A. Pulmonary artery sling: a rare cause of tracheomalacia in the adult. J Bronchology Interv Pulmonol. 2011 Jul;18(3):278-80. [CrossRef] [PubMed]
3. Ganesh V, Hoey ET, Gopalan D. Pulmonary artery sling: an unexpected finding on cardiac multidetector CT. Postgrad Med J. 2009 Mar;85(1001):128. [CrossRef] [PubMed] 

Cite as: Agrawal A, Cohen SL, Shah R, Talwar A. Medical image of the week: pulmonary artery sling. Southwest J Pulm Crit Care. 2017;15:160-61. doi: https://doi.org/10.13175/swjpcc116-17 PDF

September 2017 Imaging Case of the Month

Michael B. Gotway, MD

Department of Radiology

Mayo Clinic Arizona

Scottsdale, Arizona USA 

Clinical History: A 48-year-old woman with no previous medical history presented with complaints of intermittent cough persisting several months following a recent upper respiratory tract infection. No hemoptysis was noted.

Physical examination was largely unremarkable and the patient’s oxygen saturation was 98% on room air. Upon close inspection, the right thorax appeared slightly asymmetrically smaller than the left.

Laboratory evaluation was unremarkable. Quantiferon testing for Mycobacterium tuberculosis was negative, and testing for coccidioidomycosis was unrevealing. Frontal and lateral chest radiography (Figure 1) was performed.

Figure 1. Frontal (A) and lateral (B) chest radiography

Which of the following statements regarding the chest radiograph is most accurate? (Click on the correct answer to proceed to the second of nine pages)

1. The chest radiograph shows asymmetric reticulation and interlobular septal thickening
2. The chest radiograph shows bilateral reticulation associated with decreased lung volumes
3. The chest radiograph shows large lung volumes
4. The chest radiograph shows multifocal consolidation and pleural effusion
5. The chest radiograph shows small cavitary pulmonary nodules

Cite as: Gotway MB. September 2017 imaging case of the month. Southwest J Pulm Crit Care. 2017;15(3):104-13. doi: https://doi.org/10.13175/swjpcc109-17 PDF 

July 2016 Imaging Case of the Month

Michael B. Gotway, MD

Department of Radiology

Mayo Clinic Arizona

Scottsdale, AZ USA

Imaging Case of the Month CME Information  

Members of the Arizona, New Mexico, Colorado and California Thoracic Societies and the Mayo Clinic are able to receive  0.25 AMA PRA Category 1 Credits™. Completion of an evaluation form is required to receive credit and a link is provided on the last panel of the activity.

0.25 AMA PRA Category 1 Credit(s)™

Estimated time to complete this activity: 0.25 hours

Lead Author(s): Michael B. Gotway, MD. All Faculty, CME Planning Committee Members, and the CME Office Reviewers have disclosed that they do not have any relevant financial relationships with commercial interests that would constitute a conflict of interest concerning this CME activity. 

Learning Objectives:
As a result of this activity I will be better able to:    

1. Correctly interpret and identify clinical practices supported by the highest quality available evidence.
2. Will be better able to establsh the optimal evaluation leading to a correct diagnosis for patients with pulmonary, critical care and sleep disorders.
3. Will improve the translation of the most current clinical information into the delivery of high quality care for patients.
4. Will integrate new treatment options in discussing available treatment alternatives for patients with pulmonary, critical care and sleep related disorders.

Learning Format: Case-based, interactive online course, including mandatory assessment questions (number of questions varies by case). Please also read the Technical Requirements.

CME Sponsor: University of Arizona College of Medicine at the Arizona Health Sciences Center.

Current Approval Period: January 1, 2015-December 31, 2016

Financial Support Received: None.

Clinical History: An 18-year-old non-smoking man with a previous diagnosis of Ehlers-Danlos syndrome presented with mild shortness of breath and new cough. Physical examination was normal. The patient was afebrile.

Laboratory data were remarkable except for a mildly elevated white blood cell count of 11 x 10⁹ cells/L. Serum chemistries were within normal limits. Oxygen saturation on room air was 97%.

Frontal chest radiography (Figure 1) was performed.

Figure 1. Frontal chest radiography

A previous comparison chest radiograph from 3 years earlier (Figure 2) is shown as well.

Figure 2. Frontal and lateral chest radiography from 3 years earlier.

 Which of the following statements regarding the chest radiograph is most accurate? (Click on the correct answer to proceed to the second of seven panels)

1. The frontal chest radiograph shows new bilateral small nodular opacities
2. The frontal chest radiograph shows new focal opacity in the lateral right base 
3. The frontal chest radiograph shows new mediastinal lymphadenopathy
4. The frontal chest radiograph shows no focal abnormalities or change from prior
5. The frontal chest radiograph shows pneumothorax

Cite as: Gotway MB. July 2016 imaging case of the month. Southwest J Pulm Crit Care. 2016:13(1):15-26. doi: http://dx.doi.org/10.13175/swjpcc061-16 PDF

Medical Image of the Week: Bochdalek Hernia

Figure 1. PA (A) and lateral (B) chest radiograph demonstrating a lobulated homogenous opacity in the  posterior left lung base-blue arrows.

Figure 2. Chest CT (axial image) demonstrating fat-containing opacity consistent with a Bochdalek hernia- red arrow.

A 61 year-old man presented for an evaluation of a nonproductive cough. He has a history of well-controlled asthma, allergic rhinitis and nasal polyposis, hypertension, gastro-esophageal reflux and obstructive sleep apnea. The ACE inhibitor used to treat hypertension was discontinued. The physical exam was unremarkable. Pulmonary function testing was normal.

A PA and lateral chest radiograph was performed and revealed an abnormal contour of the left hemidiaphragm with a large lobulated opacity (Figure 1- blue arrows). Computed chest tomography revealed the lobulated opacity in the left lower lobe contained fat and was consistent with a Bochdalek hernia (Figure 2).

Congenital diaphragmatic hernia is a major malformation in newborns and in the perinatal period. The diagnosis of congenital diaphragmatic hernia in adults is rare. There are three types of congenital diaphragmatic hernias: posterolateral (Bochdalek) diaphragmatic hernia, subcostosternal (Morgagni) hernia and esophageal hiatal hernia. The Bochdalek diaphragmatic hernia is the result of a congenital diaphragmatic defect in the posterior costal part of the diaphragm in the region of 10th and 11th ribs, which allows free communication between the thoracic and abdominal cavity. The defect is usually found at the left side (90%) but may occur on the right side, where the liver often prevents detection.

A review of 173 adult patients with Bochdalek hernias revealed several important features:  55% of patients were male with an average age of 40 years, the hernia defect was located on the left side in 78% of patients and most patients were symptomatic (1,2). The most common presenting symptoms were pain or pressure in the chest or abdomen and obstruction. Pulmonary symptoms occurred in 37% of patients in this review. Of note, patients with Bochdalek hernias can develop symptoms precipitated by factors that increase intra-abdominal pressure and failure to promptly treat a symptomatic Bochdalek hernia may lead to bowel strangulation. A chest CT is an excellent diagnostic study, as a Bochdalek hernia can be difficult to appreciate on a chest radiograph (3).

Management of a Bochdalek hernia includes reducing the abdominal contents and repairing the defect through a laparotomy or thoracotomy. Successful laparoscopic and thoracoscopic repairs of Bochdalek hernias have both been described.

Muna Omar, M.D. and Linda Snyder, M.D.

Pulmonary, Critical Care, Sleep and Allergy Medicine

Banner University Medical Center-Tucson

Tucson, AZ USA

References

1. Brown SR, Horton JD, Trivette E, Hofmann LJ, Johnson JM. Bochdalek hernia in the adult: demographics, presentation, and surgical management. Hernia. 2011 Feb;15(1):23-30. [CrossRef] [PubMed]
2. Bianchi E, Mancini P, De Vito S, Pompili E, Taurone S, Guerrisi I, Guerrisi A, D'Andrea V, Cantisani V, Artico M. Congenital asymptomatic diaphragmatic hernias in adults: a case series. J Med Case Rep. 2013 May 13;7:125. [CrossRef] [PubMed]
3. Sandstrom CK, Stern EJ. Diaphragmatic hernias: a spectrum of radiographic appearances. Curr Probl Diagn Radiol. 2011 May-Jun;40(3):95-115. [CrossRef] [PubMed]

Cite as: Omar M, Snyder L. Medical image of the week: Bochdalek hernia. Southwest J Pulm Crit Care. 2016 Jun;12(6):203-4. doi: http://dx.doi.org/10.13175/swjpcc031-16 PDF

Medical Image of the Week: Persistent Left Superior Vena Cava

Figure 1. Left subclavian central line courses in a left paramediastinal location.

Figure 2. Axial CT image of the chest shows a vessel coursing lateral to the aortic arch consistent with a persistent left superior vena cava.

A 19 year old man with acute lymphocytic leukemia was admitted to the ICU with septic shock. Due to areas of cellulitis on the right side of the chest and neck and femoral venous thrombi, a left subclavian central access was attempted. The chest x-ray obtained after line placement is shown below (Figure 1). Blood gas done from the line was consistent with venous blood and venous tranduction was seen. A CT of the chest (Figure 2) confirmed the diagnosis of persistent left superior vena cava (PLSVC).

A persistent left superior vena cava is the most common congenital thoracic venous anomaly, seen in 0.3-0.5% of the population (1). Incidence is increased in patients with congenital heart disease to 5%. In most patients a right sided SVC is also present; hence the term SVC duplication has also been used. Embryologically a PLSVC is formed when the left anterior cardinal vein is not obliterated during fetal development to form the ‘Ligament of Marshall’. It usually drains in to the coronary sinus.

Diagnosis is usually made incidentally on CT scan, echocardiography or,like in our case, after a left sided central access. Practically, its presence may complicate left sided central access including Swan Ganz Catheter placement, implantable cardioverter defibrillator (ICD) placement and during cardiac surgery including during administration of retrograde cardioplegia or cardiac transplant.

Emad Hammode MD¹, Khaled Hamed MD¹, Mohammad Hudeeb MD¹, Veronica Arteaga MD²

¹Department of Internal Medicine

²Department of Thoracic Imaging

University of Arizona Medical Canter

Tucson, AZ

Reference

1. Povoski SP, Khabiri H. Persistent left superior vena cava: review of the literature, clinical implications, and relevance of alterations in thoracic central venous anatomy as pertaining to the general principles of central venous access device placement and venography in cancer patients. World J Surg Oncol. 2011;9:173. [CrossRef] [PubMed]

Reference as: Hammode E, Hamed K, Hudeeb M, Arteaga V. Medical image of the week: persistent left superior vena cava. Southwest J Pulm Crit Care. 2014;9(4):242-3. doi: http://dx.doi.org/10.13175/swjpcc132-14 PDF

Medical Image of the Week: Aortic Ring

Figure 1. Post-intubation chest radiograph showing a widened right paratracheal stripe (arrow).

A 78 year old man presented with altered mental status and was found to have an intraventricular hemorrhage. He was intubated for airway protection. On the post-intubation chest radiograph (Figure 1), the patient was noted to have a widening of the right paratracheal stripe.

A CT chest (Figure 2) was obtained to characterize this finding and revealed an aortic ring which encircles the trachea and esophagus.

Figure 2. Axial (Panel A) and coronal (Panel B) of thoracic CT soft tissue windows showing the aortic ring.

Vascular rings are uncommon congenital abnormalities, accounting for approximately 1% of congenital heart disease. Complete vascular rings can occur with a right aortic arch with a ligamentum arteriosum or with a double aortic arch, such as with our patient (1). This ring can cause airway compression, stridor, esophageal compression, or no symptoms at all. As the embryo develops, the left fourth pharyngeal arch normally persists to become the aortic arch while the right fourth pharyngeal arch regresses. If both fourth pharyngeal arches persist, a right aortic arch can form and surround the trachea and esophagus (2).

Candy Wong MD¹, Tammer Elaini MD², Soyoung Park MD², Scott Rosen MD², and Sai Parthasarathy MD¹

¹Division of Pulmonary, Allergy, Critical Care, and Sleep Medicine. Department of Medicine

²Department of Medicine

University of Arizona

Tucson, AZ

References

1. Turner A, Gavel G, Coutts J. Vascular rings-presentation, investigation and outcome. Eur J Pediatr. 2005;164(5):266-70. [CrossRef] [PubMed]
2. Vatish J, McCarthy R, Perris R. A double aortic arch presenting in the 7^th decade of life. J Surg Case Rep. 2013.(10):pii:rjt081. [CrossRef] [PubMed]

Reference as: Wong C, Elaini T, Park S, Rosen S, Parthasarathy S. Medical image of the week: aortic ring. Southwest J Pulm Crit Care. 2014;9(4):121-2. doi: http://dx.doi.org/10.13175/swjpcc130-14 PDF

Medical Image of the Week: Partial Anomalous Pulmonary Venous Return

Figure 1. Chest radiograph status post left internal jugular central line placement with arrow pointing to tip of catheter.

   

Figure 2. Axial (Panel A) and coronal (Panel B) views of contrast CT chest showing anomalous pulmonary venous return (yellow arrow). Middle Panel: Video of selected axial sections. Lower Panel: Video of selected coronal sections.

A 69 year old woman presented with septic shock secondary to a urinary tract infection. A chest radiograph (Figure 1) done after uneventful placement of a left internal jugular central line showed  aberrant position of the catheter.

Review of a past contrast-enhanced CT chest (Figure 2) revealed an anomalous pulmonary venous return with a pulmonary vein draining to the brachiocephalic vein.

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital defect which results in a left-to-right shunt. The prevalence was 0.1% in one retrospective study of 45,538 contrast-enhanced chest CT scans (1). Diagnosis can be made with echocardiography, angiography, right heart catheterization, or computed tomography. PAPVR is traditionally associated with atrial septal defects, and patients are often asymptomatic. Clinical manifestations occur when there is significant shunting and include syncope, right heart failure, and pulmonary hypertension (2).

Candy Wong MD¹; Tammer Elaini MD²; Naser  Mahmoud MD¹, and Josh Malo MD¹

¹Division of Pulmonary, Allergy, Critical Care, and Sleep Medicine. Department of Medicine.

²Department of Medicine

University of Arizona

Tucson, AZ

References

1. Ho M, Bhalla S, Bierhals A,Gutierrez F. MDCT of partial anomalous pulmonary venous return (PAPVR) in adults. J Thorac Imaging. 2009;24(2):89-95. [CrossRef] [PubMed]
2. Kivisto S, Hanninen H, Holmstrom M. Partial anomalous pulmonary venous return and atrial septal defect in adult patients detected with 128-slice multidetector computed tomography. J Cardiothorac Surg. 2011;6:126. [CrossRef] [PubMed]

Reference as: Wong C, Elaini T, Mahmoud N, Malo J. Medical image of the week: partial anomalous pulmonary venous return. Southwest J Pulm Crit Care. 2014;9(4):219-20. doi: http://dx.doi.org/10.13175/swjpcc129-14 PDF

Medical Image of the Week: Scimitar Syndrome

Figure 1. Axial CT in lung windows at the level of the right atrium shows a dilated anomalous vein (arrow) coursing in close proximity to the major fissure (star).

Figure 2. Coronal CT in soft tissue windows at the level of the right hemi diaphragm shows evidence of post surgical repair with the anomalous vein draining into the left atrium (arrow).  Note the right hemi-diaphragm is elevated suggesting some degree of pulmonary hypoplasia.

A 38 year-old woman presented for evaluation of palpitations and chronic progressive dyspnea on exertion accompanied by chest tightness and fatigue. Chest radiograph was normal except for low lung volumes. An echocardiogram revealed normal left ventricular size and function with an ejection fraction of 60%, normal right ventricular size and function and moderate tricuspid insufficiency with an estimated right ventricular systolic pressure of 36 mm Hg plus central venous pressure and a mildly enlarged right atrium. Computed tomography (CT) of the heart with contrast showed normal coronary arteries, enlarged right atrium and partial anomalous pulmonary venous return from the right lung to the inferior vena cava (IVC) (Figure 1). Cardiac catheterization with selective angiography confirmed anomalous pulmonary venous drainage from the right upper and right lower lobe to the hepatic portion of the inferior vena cava with obstruction (8 mm Hg gradient between the anomalous vein and the right atrium). The calculated pulmonary to systemic flow ratio (Qp:Qs) was 1.3:1. The pulmonary vascular resistance was 7.6 Woods units and the mean pulmonary artery pressure was 24 mmHg.  The diagnosis of Scimitar syndrome was made.

The patient underwent surgical repair of partial anomalous pulmonary drainage and pulmonary vein stenosis as well as ligation of an aortopulmonary collateral artery found intraoperatively. The anomalous pulmonary vein was divided from the IVC, the caval end was oversewn and the anomalous vein was anastomosed to the left atrium with a CorMatrix patch (Figure 2). The patient recovered from surgery, however, her symptoms continued and Doppler pattern on a follow-up transthoracic echocardiogram suggested residual obstruction at the site of anastomosis to the left atrium.  Catheterization confirmed obstruction of the anastomosed vein at the level of insertion into the left atrium.  The patient underwent repeat sternotomy and repair of pulmonary vein obstruction using a CorMatrix patch and sutureless reconstruction of the right-sided pulmonary vein obstruction.  She recovered from this procedure with some improvement of her symptoms.

Scimitar syndrome is a rare congenital anomaly characterized by partial anomalous pulmonary venous connection of the right pulmonary veins to the IVC, anomalous systemic arterial supply to the right lung and a variable degree of right lung hypoplasia with or without sequestration (1).  Partial anomalous pulmonary venous return (PAPVR) is an uncommon congenital anomaly and accounts for less than 1% of congenital heart lesions. Scimitar syndrome comprises 3-5% of PAPVR (2). The term refers to the distinctively shaped vein, which resembles a short, curved Turkish sword called a scimitar (Figure 2). Most cases present in infancy and childhood and diagnosis in adulthood is quite rare. In adults, the clinical presentation is variable and the lesion may be discovered incidentally or patients can present with dyspnea, pulmonary hypertension and recurrent right-sided lung infection (1). The treatment of isolated PAPVR in adults is controversial due to rare occurrence of the disease, complexity of surgical repair and risk of re-stenosis. Surgical repair involves re-implantation of the anomalous vein into the left atrium and can be a complex and difficult procedure, however it can be accomplished with low morbidity and mortality at specialized centers (3). Thrombosis or stenosis of the scimitar vein is a serious complication of surgery. Our patient demonstrates some of the challenges in diagnosis and treatment and illustrates some of the post-operative complications of this rare disease.

Jamie Colombo, DO¹

Linda Snyder, MD²

Veronica Arteaga, MD³

Daniela Lax, MD⁴

¹Department of Pediatrics

²Department of Medicine, Division of Pulmonary/Critical Care Medicine

³Department of Medical Imaging

⁴Department of Pediatrics, Division of Pediatric Cardiology

University of Arizona

Tucson, AZ

References

1. Yehia BR, Bachmann JM, Traill TA. Scimitar syndrome: a rare cause of dyspnea in adults. South Med J. 2010;103:578-580. [CrossRef] [PubMed]
2. Haest RJ, van den Berg CJ, Goei R, Baur LH. Scimitar syndrome; an unusual congenital abnormality occasionally seen in adults. Int J Cardiovasc Imaging. 2006;22:565-568. [CrossRef] [PubMed]
3. Majdalany DS, Phillips SD, Dearani JA, Connolly HM, Warnes CA. Isolated partial anomalous venous connections in adults: twenty-year experience. Congenit Heart Dis. 2010;5:537-545. [CrossRef] [PubMed]

Reference as: Colombo J, Snyder L, Arteaga V, Lax D. Medical image of the week: scimitar syndrome. Southwest J Pulm Crit Care. 2014;9(2):137-9. doi: http://dx.doi.org/10.13175/swjpcc111-14 PDF