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Hypogenetic right lung with partial anomalous pulmonary venous return and accessory diaphragm: a case of “scimitar lung”
Affiliations- Department of Biomedical Sciences, Cooper Medical School of Rowan University, Camden, New Jersey, United States
- Rowan University, Glassboro, New Jersey, United States
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Abstract
Partial anomalous pulmonary venous return (PAPVR) is a rare congenital cardiovascular
condition in which some of the pulmonary veins drain into the systemic
circulation. We report on the cadaveric dissection of a 71-year-old Caucasian male
donor who died of chronic obstructive pulmonary disease with hypertension. We
noted a faint incisional scar on the thorax extending from the parasternal region
at the 4th intercostal level to the midaxillary line. Since the straight-line incision
followed the ribs and the scar was quite faint, surgery probably occurred when
the donor was young. We also observed numerous surgical interventions of the
heart, lungs, and vasculature to correct various defects.
The morphology of the heart was normal, but was shifted more to the right
side. An atrial septal defect (ASD) was closed with sutures. The right superior
pulmonary vein that drained into the superior vena cava (SVC) was ligated close
to the SVC and the right inferior, left superior, and inferior pulmonary veins all
drained directly into the left atrium. We noticed a dilated coronary sinus entering
the right atrium adjacent to the ASD; the ostium of the coronary sinus noticeably
lacked the normal valve-like structure. We initially thought the right lung was
a “horseshoe” lung, but realised that it was a “hypogenetic” lung with PAPVR and
an accessory diaphragm. Compared to the left, the right secondary bronchi were
much narrower and branched uncharacteristically, as seen in hypogenetic lung
syndrome. The inferior lobe was highly disorganised, severely hypoplastic, and
exhibited uncharacteristic morphology. The superior bronchopulmonary segment
was markedly hypoplastic. The posterior and medial basal segments were not
only hypoplastic and slender, but also extended like a tail to the left pulmonary
cavity behind the heart/pericardium and in front of the oesophagus and aorta.
The right lung, though hypoplastic, demonstrated patent bronchi and the lobes
were inflatable. Based on the hypogenetic lung and PAPVR, we conclude that the
donor exhibited ‘scimitar’ lung.
Abstract
Partial anomalous pulmonary venous return (PAPVR) is a rare congenital cardiovascular
condition in which some of the pulmonary veins drain into the systemic
circulation. We report on the cadaveric dissection of a 71-year-old Caucasian male
donor who died of chronic obstructive pulmonary disease with hypertension. We
noted a faint incisional scar on the thorax extending from the parasternal region
at the 4th intercostal level to the midaxillary line. Since the straight-line incision
followed the ribs and the scar was quite faint, surgery probably occurred when
the donor was young. We also observed numerous surgical interventions of the
heart, lungs, and vasculature to correct various defects.
The morphology of the heart was normal, but was shifted more to the right
side. An atrial septal defect (ASD) was closed with sutures. The right superior
pulmonary vein that drained into the superior vena cava (SVC) was ligated close
to the SVC and the right inferior, left superior, and inferior pulmonary veins all
drained directly into the left atrium. We noticed a dilated coronary sinus entering
the right atrium adjacent to the ASD; the ostium of the coronary sinus noticeably
lacked the normal valve-like structure. We initially thought the right lung was
a “horseshoe” lung, but realised that it was a “hypogenetic” lung with PAPVR and
an accessory diaphragm. Compared to the left, the right secondary bronchi were
much narrower and branched uncharacteristically, as seen in hypogenetic lung
syndrome. The inferior lobe was highly disorganised, severely hypoplastic, and
exhibited uncharacteristic morphology. The superior bronchopulmonary segment
was markedly hypoplastic. The posterior and medial basal segments were not
only hypoplastic and slender, but also extended like a tail to the left pulmonary
cavity behind the heart/pericardium and in front of the oesophagus and aorta.
The right lung, though hypoplastic, demonstrated patent bronchi and the lobes
were inflatable. Based on the hypogenetic lung and PAPVR, we conclude that the
donor exhibited ‘scimitar’ lung.
Keywords
scimitar lung, congenital cardiovascular anomaly, partial anomalous pulmonary venous return (PAPVR), partial anomalous pulmonary venous connection (PAPVC)
About this articleTitle
Hypogenetic right lung with partial anomalous pulmonary venous return and accessory diaphragm: a case of “scimitar lung”
Journal
Issue
Article type
Case report
Pages
980-987
Published online
2022-11-29
Page views
932
Article views/downloads
573
DOI
Pubmed
Bibliographic record
Folia Morphol 2023;82(4):980-987.
Keywords
scimitar lung
congenital cardiovascular anomaly
partial anomalous pulmonary venous return (PAPVR)
partial anomalous pulmonary venous connection (PAPVC)
Authors
C. Melovitz-Vasan
A. White
S. Huff
N. Vasan
References (37)- Ahamed MF, Al Hameed F. Hypogenetic lung syndrome in an adolescent: imaging findings with short review. Ann Thorac Med. 2008; 3(2): 60–63.
- Canter C, Martin T, Spray T, et al. Scimitar syndrome in childhood. Am J Cardiol. 1986; 58(7): 652–654.
- Chassinat R. Observation d’anomalies anatomiques remarquables de l’appareil circulatoire, aved hépatocèle congénitale, n’ayant donné lieu pendant la vie à aucun symptôme particulier: suivie de réflexions. II. Archives générales de médecine, Paris 1836: 80–91.
- Cooper G. Case of malformation of the thoracic viscera consisting of imperfect development of the right lung and transposition of the heart. London Medical Gazette. 1836; 18: 600–602.
- Currarino G, Williams B. Causes of congenital unilateral pulmonary hypoplasia: a study of 33 cases. Pediatr Radiol. 1985; 15(1): 15–24.
- Darling RC, Craig JM, Rothney WB. Total pulmonary venous drainage into the right side of the heart; report of 17 autopsied cases not associated with other major cardiovascular anomalies. Lab Invest. 1957; 6(1): 44–64.
- Daves ML. Cardiac roentgenology: shadows of the heart. Year-Book, Chicago, Ill 1981: 274–281.
- Davis WS, Allen RP. Accessory diaphragm. Duplication of the diaphragm. Radiol Clin North Am. 1968; 6(2): 253–263.
- Demos TC, Posniak HV, Pierce KL, et al. Venous anomalies of the thorax. AJR Am J Roentgenol. 2004; 182(5): 1139–1150.
- Dikensoy O, Kervancioglu R, Bayram NG, et al. Horseshoe lung associated with scimitar syndrome and pleural lipoma. J Thorac Imaging. 2006; 21(1): 73–75.
- Dillman JR, Yarram SG, Hernandez RJ. Imaging of pulmonary venous developmental anomalies. AJR Am J Roentgenol. 2009; 192(5): 1272–1285.
- Felson B. Chest roentgenology. Philadelphia, Saunders 1973: 81–92.
- Folger GM. The scimitar syndrome. Anatomic, physiologic, developmental and therapeutic considerations. Angiology. 1976; 27(6): 373–407.
- Frank JL, Poole CA, Rosas G. Horseshoe lung: clinical, pathologic, and radiologic features and a new plain film finding. AJR Am J Roentgenol. 1986; 146(2): 217–226.
- Frydrychowicz A, Landgraf B, Wieben O, et al. Images in Cardiovascular Medicine. Scimitar syndrome: added value by isotropic flow-sensitive four-dimensional magnetic resonance imaging with PC-VIPR (phase-contrast vastly undersampled isotropic projection reconstruction). Circulation. 2010; 121(23): e434–e436.
- Gaillard F, Weerakkody Y. Scimitar syndrome (lungs). Reference article, Radiopaedia.org. https://radiopaedia.org/articles/2027 (accessed on 21 Apr 2022).
- Goo HW, Kim YH, Ko JK, et al. Horseshoe lung: useful angiographic and bronchographic images using multidetector-row spiral CT in two infants. Pediatr Radiol. 2002; 32(7): 529–532.
- Gustafson R, Warden H, Murray G, et al. Partial anomalous pulmonary venous connection to the right side of the heart. J Thorac Cardiovasc Surg. 1989; 98(5): 861–868.
- Halasz NA, Halloran KH, Liebow AA. Bronchial and arterial anomalies with drainage of the right lung into the inferior vena cava. Circulation. 1956; 14(5): 826–846.
- Hartman T. Case 9 - Horseshoe lung. In: Hartman T (ed.). Pearls and Pitfalls in Thoracic Imaging: Variants and Other Difficult Diagnoses. Cambridge University Press, Cambridge 2011: 22–23.
- Ho ML, Bhalla S, Bierhals A, et al. MDCT of partial anomalous pulmonary venous return (PAPVR) in adults. J Thorac Imaging. 2009; 24(2): 89–95.
- Ibrahim M, Burwash IG, Morton B, et al. Direct drainage of the right pulmonary veins into the coronary sinus with intact interatrial septum: a case report. Can J Cardiol. 2001; 17(7): 807–809.
- Kabbani M, Haider N, Abu-Sulaiman R. Bilateral scimitar syndrome. Cardiol Young. 2004; 14(4): 447–449.
- Kamijoh M, Itoh M, Kijimoto C, et al. Horseshoe lung with bilateral vascular anomalies: a rare variant of hypogenetic lung syndrome (scimitar syndrome). Pediatr Int. 2002; 44(4): 443–445.
- Konen E, Raviv-Zilka L, Cohen R, et al. Congenital pulmonary venolobar syndrome: spectrum of helical CT findings with emphasis on computerized reformatting. RadioGraphics. 2003; 23(5): 1175–1184.
- Mata JM, Cáceres J, Lucaya X, et al. CT of congenital malformations of the lung. Radiographics. 1990; 10(4): 651–674.
- Muthiah R. Coronary sinus atrial septal defect (unroofed coronary sinus) with total anomalous pulmonary venous connection: a case report. Case Rep Clin Med. 2017; 06(01): 1–18.
- Nazarian M, Currarino G, Webb WR, et al. Accessory diaphragm. Report of a case with complete physiological evaluation and surgical correction. J Thorac Cardiovasc Surg. 1971; 61(2): 293–299.
- Olson MA, Becker GJ. The Scimitar syndrome: CT findings in partial anomalous pulmonary venous return. Radiology. 1986; 159(1): 25–26.
- Partridge JB, Osborne JM, Slaughter RE. Scimitar etcetera: the dysmorphic right lung. Clin Radiol. 1988; 39(1): 11–19.
- Petullà M, Comito L, Settino V, et al. Scimitar syndrome (or Halasz Syndrome): description of a case originating in adulthood. J Radiol Rev. 2020; 7(6).
- Silawal S, Essing T, Schaupp C, et al. Rare malformations associated with partial anomalous pulmonary venous return: a cadaveric case report. Folia Morphol. 2022; 81(2): 526–531.
- Takahashi M, Murata K, Yamori M, et al. Horseshoe lung: demonstration by electron-beam CT. Br J Radiol. 1997; 70(837): 964–966.
- Tomisawa M, Goto M, Kimpara K, et al. Accessory diaphragm: report of a case associated with atrial septal defect. J Pediatr Surg. 1974; 9(2): 223–226.
- Tosun A, Leblebisatan S. Congenital pseudohorseshoe lung associated with scimitar syndrome. Iran J Radiol. 2012; 9(2): 99–102.
- van den Hoven AT, Chelu RG, Duijnhouwer AL, et al. Partial anomalous pulmonary venous return in Turner syndrome. Eur J Radiol. 2017; 95: 141–146.
- Woodring JH, Howard TA, Kanga JF. Congenital pulmonary venolobar syndrome revisited. Radiographics. 1994; 14(2): 349–369.
