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	Figure S1. Development of human lungs. (A) Ventral diverticulum of the foregut lined by endodermal epithelium invested by splanchnic mesenchyme at day 26. (B, C) Right and left lung buds develop within the next 2 days. (D) Lateral ingrowths of the mesoderm meet to form the tracheoesophageal septum, separating the respiratory and esophageal portions. (E) By days 32-34, the five lobar bronchi have appeared. (F) The pseudoglandular period extends from the 5th to the 16th week of gestation and primarily relates to development of the bronchial tree. (Taken, with permission, from Fraser RS, Müller NL, Colman N, Paré PD. Fraser and Paré's Diagnosis of Diseases of the Chest. 4th ed. Philadelphia, Pa: Saunders, 1999.)

	Figure S2. Development of pulmonary arteries. (A) Aortic arches 3, 4, and 6 at 6 weeks. (B) Aortic arches at 7 weeks. Broken lines indicate segments that disappear. (C) Arrangement of vessels at 8 weeks. (Taken, with permission, from Fraser RS, Müller NL, Colman N, Paré PD. Fraser and Paré's Diagnosis of Diseases of the Chest. 4th ed. Philadelphia, Pa: Saunders, 1999.)

	Figure S3. Development of pulmonary veins. (A) The lung buds drain via the splanchnic plexus into the umbilical and cardinal veins. (B) The pulmonary veins now also communicate with the left atrium. (C) With the disappearance of the primitive systemic connections, complete flow is into the left atrium. (Taken, with permission, from Fraser RS, Müller NL, Colman N, Paré PD. Fraser and Paré's Diagnosis of Diseases of the Chest. 4th ed. Philadelphia, Pa: Saunders, 1999.)


a.	b.

c.	d.
Figure S4. Bronchial atresia in an 18-year-old woman with repeated infections dating back to childhood. (a) Chest radiograph at presentation demonstrates a slight shift of the mediastinum to the left due to overinflation in the right upper zone with accompanying underperfusion. A large cavity is in the expected position of the right middle lobe. A solid masslike density resides in the superior aspect of the right hilum. (b) A posterior ventilation-perfusion scan obtained 3 years earlier shows a matching defect in the right upper lung. (c, d) CT scans obtained at two different levels show large fluid-filled masses representing markedly dilated upper lobe bronchi. A right upper lobectomy was planned, but because of significant scar tissue, dissection of the pulmonary arteries could not be done. A right pneumonectomy was performed. Atresia of the right upper lobe bronchus was confirmed.


a.

b.	c.

d.	e.
Figure S5. Bronchial atresia. (a) A routine preoperative study in a 29-year-old male patient was interpreted as showing localized emphysema in the right upper lobe. (b) Close-up view demonstrates a cystic space with a small amount of fluid. In addition, there are peculiar somewhat hairpin-shaped linear opacities that contain air. The patient was admitted 4 years later with acute pancreatitis. (c) Frontal radiograph shows the former hairpin loops now to be fluid filled and the central cavity to be completely opaque. These represent the poststenotic dilated bronchi. (d, e) CT scans obtained at different levels while the patient was in the intensive care unit show overinflation of the right upper lobe via collateral air drift. The vessels are correspondingly small and somewhat distorted. The tubular structures represent dilated bronchi.


a.
Figure S6. Congenital cystic adenomatoid malformation in a 31-year-old woman with a history of recurrent pneumonia. (a) CT study demonstrates air and fluid within loculated spaces as well as adjacent air space disease, likely pneumonia. The patient underwent a left-lower-lobe lobectomy. (b) The specimen shows the presence of multiple cystic spaces of varying size. (Courtesy of Tomas Franquet, MD, Barcelona, Spain.)
	b.


a.	b.
Figure S7. Coarctation of right main pulmonary artery. (a) Frontal radiograph of a 30-year-old asymptomatic male patient shows a small hyperlucent right lung. There was no air trapping on the exhalation view (not shown). The vessels in the right lung are small, presumably because of systemic origin. A perfusion scan (not shown) demonstrated absence of perfusion to the right lung. (b) Digital right pulmonary angiogram demonstrates narrowing (coarctation) of the right main pulmonary artery (arrows). Distal branching was normal except that the branches were reduced in caliber. The left side was normal.


a.	b.
Figure S8. Interruption (absence) of left pulmonary artery. (a) Frontal radiograph of a 19-year-old woman demonstrates findings similar to those in Figure 7. The left lung is small and hyperlucent with diminutive vessels. There was no air trapping on the exhalation view (not shown). There is a right aortic arch, and the right lung is overinflated. (b) Anterior perfusion scan demonstrates absence of pulmonary flow to the left lung.


a.	b.
Figure S9. Anomalous drainage of left lung in a 44-year-old man. (a) Frontal radiograph shows widening of the left upper mediastinum. (b) Venous phase of a pulmonary angiogram demonstrates anomalous drainage of the entire left lung into the left brachiocephalic vein. Drainage on the right is normal.


a.	b.

c.	d.

e.	f.
Figure S10. Sequestration in a 62-year-old man. (a) Posteroanterior radiograph demonstrates a mass in the left lower lobe. (b) On the lateral view, the mass projects over the anterior aspect of one of the lower dorsal vertebrae. Bronchogenic carcinoma was suspected. (c, d) CT scans through two levels of the lesion, however, demonstrate a connection between the mass and the aorta. The connection contains some calcifications, in keeping with atherosclerosis in the supplying artery. The CT lung windows demonstrate adjacent areas of low attenuation, consistent with trapping of air entering via collateral ventilation. (e) Photomicrograph (original magnification, x10; hematoxylin-eosin stain) of calcific atherosclerotic systemic artery (arrow) with adjacent bronchus (arrowhead) and pulmonary parenchyma. (f) Photomicrograph (original magnification, x5; hematoxylin-eosin stain) of markedly fibrotic lung parenchyma with bronchiolization of cystic airspaces, many of which contain inspissated mucus.


a.	b.
Figure S11. Sequestration in a 39-year-old woman with an abnormal opacity in the left lung base on conventional radiographs (not shown). (a, b) CT scan (a) and MR image (b) demonstrate a systemic artery from the aorta supplying abnormally aerated lung in the left lower lobe.

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