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"Absent" Pulmonary Artery in One Adult and Five Pediatric Patients: Imaging, Embryology, and Therapeutic Implications

¹ Department of Pediatric Cardiology, Onassis Cardiac Surgery Center, 356 Syngrou Ave., Athens, GR 176 74, Greece.
² Department of Radiology, University of Thessalia Medical School, 22 Papakyriazi St., Larissa, GR 41221, Greece.
³ Second Department of Radiology, Eugenidion University Hospital, University of Athens, 20 Papadiamantopoulou St., Athens, GR 115 28, Greece.

View larger version (147K): [in a new window]   Fig. 1A. —6-year-old girl with right aortic arch, mirror-image branching, and left-sided "absent" pulmonary artery. Posteroanterior chest radiograph shows smaller left-sided hemithorax with absent ipsilateral pulmonary artery shadow; mediastinal shift to left, with mild elevation of left hemidiaphragm; and presence of right aortic arch.

View larger version (128K): [in a new window]   Fig. 2A. —18-month-old girl with large perimembranous ventricular septal defect, right aortic arch, and right-sided "absent" pulmonary artery. Posteroanterior chest radiograph shows smaller right lung with decreased pulmonary vascular markings, slight mediastinal shift to right and elevated right hemidiaphragm, absence of ipsilateral pulmonary artery shadow, right aortic arch, and increased vascular markings on left lung attributable to underlying ventricular septal defect.

View larger version (131K): [in a new window]   Fig. 3A. —10-day-old boy with truncus arteriosus, right aortic arch with mirror-image branching, and left-sided "absent" pulmonary artery. Posteroanterior chest radiograph shows right aortic arch with mildly decreased pulmonary vascular markings on left.

View larger version (146K): [in a new window]   Fig. 4A. —2-month-old boy with heterotaxy, dextrocardia, visceral situs inversus, single right ventricle, pulmonary atresia, total pulmonary venous return to right innominate vein, and bilateral "absent" pulmonary arteries. Posteroanterior chest radiograph shows dextrocardia with globular right heart border, prominent upper mediastinum, and decreased pulmonary vascular markings with mild hyperinflation bilaterally. Central venous catheter is in place in left superior vena cava.

View larger version (95K): [in a new window]   Fig. 1B. —6-year-old girl with right aortic arch, mirror-image branching, and left-sided "absent" pulmonary artery. Oblique axial maximum-intensity-projection image derived from set of axial helical CT images (obtained during arterial phase) reveals absence of left main pulmonary artery and aortopulmonary collaterals (arrowhead) from descending aorta coursing through hilum to reach tiny intrapulmonary vessels (arrow).

View larger version (133K): [in a new window]   Fig. 1C. —6-year-old girl with right aortic arch, mirror-image branching, and left-sided "absent" pulmonary artery. Angiogram of main pulmonary artery shows single right pulmonary artery with increased caliber, mild tortuosity of peripheral vessels, and absence of left pulmonary artery.

View larger version (149K): [in a new window]   Fig. 1D. —6-year-old girl with right aortic arch, mirror-image branching, and left-sided "absent" pulmonary artery. Pulmonary vein wedge angiogram obtained in arterial phase shows extremely hypoplastic 1- to 2-mm peripheral vessel with pulmonary arterial distribution that reaches only to hilum (arrow).

View larger version (144K): [in a new window]   Fig. 2B. —18-month-old girl with large perimembranous ventricular septal defect, right aortic arch, and right-sided "absent" pulmonary artery. Postoperative pulmonary angiogram obtained in anteroposterior projection shows single left-sided pulmonary artery with normal distribution, stenosis at its origin (arrow), and absence of right pulmonary artery.

View larger version (148K): [in a new window]   Fig. 2C. —18-month-old girl with large perimembranous ventricular septal defect, right aortic arch, and right-sided "absent" pulmonary artery. Postoperative pulmonary angiogram obtained in lateral projection shows multiple stenoses at origin (open arrow) of left pulmonary artery as well as its major branches (solid arrows).

View larger version (118K): [in a new window]   Fig. 3B. —10-day-old boy with truncus arteriosus, right aortic arch with mirror-image branching, and left-sided "absent" pulmonary artery. Angiogram of ascending aorta obtained 10 days after birth shows patency of ductus arteriosus from base of left innominate artery (open arrow) supplying intrapulmonary left pulmonary artery of normal caliber and distribution but with no extrapulmonary portion. Main pulmonary trunk originates from ascending aorta (solid arrow) and gives rise to right pulmonary artery.

View larger version (132K): [in a new window]   Fig. 3C. —10-day-old boy with truncus arteriosus, right aortic arch with mirror-image branching, and left-sided "absent" pulmonary artery. Angiogram of ascending aorta obtained at 6 months shows significant regression of connecting patent ductus arteriosus (arrow).

View larger version (126K): [in a new window]   Fig. 3D. —10-day-old boy with truncus arteriosus, right aortic arch with mirror-image branching, and left-sided "absent" pulmonary artery. Angiogram of left innominate artery obtained at 12 months shows presence of blind stump (solid arrow) with disappearance of previously patent ductus arteriosus. Superimposed aorto pulmonary collateral vessel (open arrow) is supplying parts of left upper lobe.

View larger version (136K): [in a new window]   Fig. 4B. —2-month-old boy with heterotaxy, dextrocardia, visceral situs inversus, single right ventricle, pulmonary atresia, total pulmonary venous return to right innominate vein, and bilateral "absent" pulmonary arteries. Angiogram of aortic arch shows patent right ductus arteriosus from base of right innominate artery supplying right intrapulmonary pulmonary artery and stenosis (arrow) at their junction. Large tortuous left ductus arteriosus (arrowhead) from underside of aortic arch supplies left intrapulmonary pulmonary artery. Bilateral absence of extrapulmonary pulmonary arteries is evident.

View larger version (124K): [in a new window]   Fig. 3E. —10-day-old boy with truncus arteriosus, right aortic arch with mirror-image branching, and left-sided "absent" pulmonary artery. Angiogram of left-sided modified Blalock-Taussig shunt (arrow) obtained 6 months after surgery shows mildly hypoplastic but patent intrapulmonary left pulmonary artery with normal distribution and adequate antegrade flow. Mild stenosis (arrow) at anastomotic site is seen.

View larger version (34K): [in a new window]   Fig. 5. —6-year-old girl with mirror-image right aortic arch with left ductus arteriosus from base of left innominate artery and left-sided "absent" pulmonary artery (same patient as pictured in Fig. 1A,1B,1C,1D). Diagram shows primitive (seventh fetal week) and final arch and pulmonary artery anatomy. Roman numerals denote six (I-VI) primitive aortic arches and seventh intersegmental artery (VII). Truncoaortic sac is depicted in dark gray, ventral aorta in pink, dorsal aorta in light gray, intrapulmonary pulmonary arteries in light blue, and primitive aortic arches: III in red, IV in yellow, and VI in dark blue.

View larger version (32K): [in a new window]   Fig. 6. —18-month-old girl with large perimembranous ventricular septal defect, right aortic arch with retroesophageal left subclavian artery (persistence of caudal part of left dorsal aorta and involution of left IV aortic arch) without diverticulum of Kommerell, right ductus arteriosus from underside of arch, and right-sided "absent" pulmonary artery (same patient as in Fig. 2A,2B,2C). Diagram shows primitive (seventh fetal week) and final arch and pulmonary artery anatomy. Roman numerals denote six (I-VI) primitive aortic arches and seventh intersegmental artery (VII). Truncoaortic sac is depicted in dark gray, ventral aorta in pink, dorsal aorta in light gray, intrapulmonary pulmonary arteries in light blue, and primitive aortic arches: III in red, IV in yellow, and VI in dark blue.

View larger version (32K): [in a new window]   Fig. 7. —2-month-old boy with heterotaxy, dextrocardia, visceral situs inversus, single right ventricle, total pulmonary venous return to right innominate vein, pulmonary atresia, left aortic arch with normal branching, and bilateral ductus arteriosi supplying intrapulmonary right and left pulmonary arteries with bilateral absent extrapulmonary portions. Right ductus arteriosus originates from base of right innominate artery and left ductus arteriosus from underside of arch. (same patient as in Fig. 4A,4B). Diagram shows primitive (seventh fetal week) and final arch and pulmonary artery anatomy. Roman numerals denote six (I-VI) primitive aortic arches and seventh intersegmental artery (VII). Truncoaortic sac is depicted in dark gray, ventral aorta in pink, dorsal aorta in light gray, intrapulmonary pulmonary arteries in light blue, and primitive aortic arches: III in red, IV in yellow, and VI in dark blue.

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