Pediatric Radiology
Pediatr. Radiol. 8, 195-i97 (1979)
9 by Springer-Verlag1979
Common Pulmonary Vein Atresia without Anomalous Pulmonary Venous Connection C. T. D e L i s e ~, B. S c h n e i d e r , a n d M. S. B l a c k m a n Department of Radiology and Pediatrics, Crouse-Irving Memorial Hospital, Syracuse, New York and the Division of Cardiology, Department of Pediatrics, Upstate Medical Center, Syracuse, New York, USA
Abstract. C o m m o n p u l m o n a r y vein atresia w i t h o u t p u l m o n a r y v e n o u s c o n n e c t i o n is a rare f o r m of c o n genital h e a r t disease. N o c o m m u n i c a t i o n exists b e t w e e n the c o n f l u e n c e of the p u l m o n a r y v e i n s a n d the h e a r t or a m a j o r systemic vessel. A case d i a g n o s e d a n t e m o r t e m is p r e s e n t e d . C o r r e c t early diagnosis is i m p e r a t i v e as s u r g e r y m a y b e corrective.
Key words: P u l m o n a r y v e n o u s o b s t r c t i o n - A n o m a lous p u l m o n a r y v e n o u s d r a i n a g e
C o m m o n p u l m o n a r y v e i n atresia ( C P V A ) w i t h o u t a n o m a l o u s p u l m o n a r y v e n o u s c o n n e c t i o n is o n e of the rarest a n o m a l i e s of the p u l m o n a r y v e n o u s system. T h e e n t i t y was first d e s c r i b e d b y L u c a s [1]. F o u r t e e n p r e v i o u s cases have b e e n p u b l i s h e d [2]. I n all instances, the diagnosis was c o n f i r m e d at a u t o p s y ; o n l y s e v e n cases have b e e n d i a g n o s e d a n t e m o r t e m . This r e p o r t r e p r e s e n t s the fifteenth e x a m p l e of this e n t i t y to b e publiShed a n d the eighth to b e d i a g n o s e d b e f o r e death. C P V A without anomalous pulmonary venous c o n n e c t i o n is u n i f o r m l y fatal, yet if correctly diagn o s e d e a r l y a n d m a n a g e d aggressively m a y b e a m e n a b l e to surgical correction. It is for this r e a s o n that we s u b m i t this report.
from another hospital at seven hours of age. Blood gases on arrival revealed profound acidosis and hypoxemia refractory to treatment; PO2 remained at 19 mm. of Hg. despite oxygen and ventilatory support. On the electrocardiogram there was a right axis at + 150 degrees and marked right ventricular hypertrophy. Chest films (Fig. 1) revealed a normal sized heart. The lungs were hyperinflated and revealed a diffuse reticular pattern consistent with an obstructing lesion distal to the pulmonary capillary bed. On physical examination, the infant was a markedly cyanotic, tachypneic female. The pulse was 160, respiratory rate 80, and systolic blood pressure 60. The lungs were clear. The heart was not enlarged, the second sound was split and accentuated; no murmurs were heard. The heart tones were of poor quality. Cardiac catheterization was performed at ten hours of age. The infant was bradycardiac and cardiac output was difficult to maintain during the procedure. Samples and pressures were obtained in all four chambers and both great vessels. Pulmonary artery pressure was systemic and there was marked right to left shunting. Biplane angiograms were performed from the left ventricle and the main pulmonary artery. The left ventricle, aortic valve and ascending aorta were normal. There was retrograde filling of the ascending aorta on the main pulmonary artery angiogram suggestive of a markedly diminished left ventricular output. The pulmonary artery angiogram (Fig. 2) revealed visualization of a CPV, but no systemic or cardiac connection. A diagnosis of CPVA was made by exclusion of hypoplastic left heart, and total anomalous pulmonary venous return. Following catheterization plans were made for corrective surgery. Her condition continued to deteriorate rapidly, she developed bilateral pneumothorax and had profound uncorrectable acidosis and hypotension and was not considered to be a viable surgical candidate, death occurred at 16 hours of age.
Autopsy Case Report L.S. was a term 2400 gram white female born to a 32 year old gravida 7 para 5 mother. The patient was noted to be cyanotic shortly after birth and was transferred to our special care nursery
1 Present Address: Champlain Valley Hospital, Plattsburg, New York
E x a m i n a t i o n of the h e a r t r e v e a l e d a n atrial septal defect, 5 cm in d i a m e t e r . T h e d u c t u s was p a t e n t . T h e right a n d left p u l m o n a r y veins j o i n e d t o g e t h e r to f o r m a c o m m o n c h a n n e l which did n o t c o m m u n i c a t e with either atrium. T h e azygos s y s t e m was dilated. N o c o m m u n i c a t i o n b e t w e e n the p u l m o n a r y v e n o u s
0301-0449/79/0008/0195/$01.00
196
C.T. DeLise et al.: CommonPulmonaryVein Atresia
Fig. la and b. AP and lateral chest showthat the heart is normal in size. The lungs are over-inflated and reveal a diffuse reticular pattern bilaterally. Fluid is evident in the pleural fissure
Fig. 2. AP angiogram 13 secondsafter the main pulmonary artery injection. Diffuse opacificationof peripheral pulmonary veinspersists and a small CPV can be seen in normal position behind the left atrium ( - ---~).It is not dilated. Faint visualizationof the azygosvein can be appreciated (+ ---~).Additional filmingfor a total of 30 secondsdemonstrated no cardiacchamber opacificationand no other systemic venousvisualization. The opacifiedpulmonaryveins gradually diminished in densityduring the 30 secondillming sequence
system and the heart or systemic veins could be identified. Peribronchial collaterals could not be identified.
Discussion Embryologically, if an obstruction to pulmonary venous return occurs because of failure of incorporation of the CPV into the left atrium, several possible mechanisms to compensate for the defect may be operative depending on the age of the embryo. If obstruction occurs early when primitive systemic venous pathways have not yet regressed, pulmonary venous return may be diverted into one or more of these venous systems. Persistent blood flow through these collateral pathways prevents their normal re-
gression and one of the more c o m m o n types of anomalous pulmonary venous drainage results. If incorporation of the C P V into the left atrium is incomplete and the ostium is stenotic, cor triatriatum is encountered. It has been suggested that C P V A without anomalous pulmonary venous connection results if the failure of incorporation of the C P V into the left atrium occurs somewhat later in embryologic life and the primitive venous pathways have undergone regression. The blood returning from the lungs, therefore, has no well developed path of exodus from the c o m m o n pulmonary vein [1]. The fetus is able to tolerate this severe obstruction to pulmonary venous return because the lungs in utero receive only 8% of the total cardiac output. The little venous return to the CPV can be effectively directed through the peribronchial venous plexuses
C. T. DeLise et al.: Common Pulmonary Vein Atresia
which anastomose with the pulmonary veins to drain the centrolobular and juxtabronchial alveoli [3, 4]. These peribronchial venous plexuses drain into systemic veins at the hilum of the lung and eventually enter the azygos and hemi-azygos systems. Visualization of the azygos vein (Fig. 2) indicates that such collateral pathways were extant in our case. Pulmonary lymphatics may also divert excessive fluid accumulation from the lung in venous obstruction [4, 5]. After birth when the lungs expand, pulmonary blood flow and venous return increase dramatically. The marginal collateral pathways developed in utero are overwhelmed and progressive clinical deterioration results in early death. To date, no infant with CPVA has survived; although successful surgical anastomosis of the CPV to the left atrium has been performed. Extreme acidosis and hypoxia make these infants poor surgical candidates, yet, early diagnosis and surgery under hypothermic arrest remains their only hope for survival.
197
References 1. Lucas, R.V., Jr., Woolfrey, B.V., Anderson, R.C., Lester, R. G., Edwards, J. E.: Atresia of the common pulmonary vein. Pediatrics 29, 729 (1962) 2. Ledbetter, M. K., Wells, D. H., Connors, D. M.: Common pulmonary vein atresia. Am. Heart J. 96, 580 (1978) 3. Roberson, B.: Microangiography of the lung in infancy and childhood. Propius, 1973 4. Liebow, A. A.: The bronchopulmonary venous collateral circulation with special reference to emphysema. Am. J. Pathol. 29,
251 (1953) 5. Rabin, E. R., Meyer, E. C.: Cardiopulmonary effects of pulmonary venous hypertension with special reference to pulmonary lymphatic flow. Circ. Res. 8, 324 (1960) Date of final acceptance: December 13, 1978 Bernard Schneider, M. D. Department of Radiology Crouse-Irving Memorial Hospital 736 Irving Avenue Syracuse, NY 13210 USA
Pediatr. Radiol. 8, 195-i97 (1979)
9 by Springer-Verlag1979
Common Pulmonary Vein Atresia without Anomalous Pulmonary Venous Connection C. T. D e L i s e ~, B. S c h n e i d e r , a n d M. S. B l a c k m a n Department of Radiology and Pediatrics, Crouse-Irving Memorial Hospital, Syracuse, New York and the Division of Cardiology, Department of Pediatrics, Upstate Medical Center, Syracuse, New York, USA
Abstract. C o m m o n p u l m o n a r y vein atresia w i t h o u t p u l m o n a r y v e n o u s c o n n e c t i o n is a rare f o r m of c o n genital h e a r t disease. N o c o m m u n i c a t i o n exists b e t w e e n the c o n f l u e n c e of the p u l m o n a r y v e i n s a n d the h e a r t or a m a j o r systemic vessel. A case d i a g n o s e d a n t e m o r t e m is p r e s e n t e d . C o r r e c t early diagnosis is i m p e r a t i v e as s u r g e r y m a y b e corrective.
Key words: P u l m o n a r y v e n o u s o b s t r c t i o n - A n o m a lous p u l m o n a r y v e n o u s d r a i n a g e
C o m m o n p u l m o n a r y v e i n atresia ( C P V A ) w i t h o u t a n o m a l o u s p u l m o n a r y v e n o u s c o n n e c t i o n is o n e of the rarest a n o m a l i e s of the p u l m o n a r y v e n o u s system. T h e e n t i t y was first d e s c r i b e d b y L u c a s [1]. F o u r t e e n p r e v i o u s cases have b e e n p u b l i s h e d [2]. I n all instances, the diagnosis was c o n f i r m e d at a u t o p s y ; o n l y s e v e n cases have b e e n d i a g n o s e d a n t e m o r t e m . This r e p o r t r e p r e s e n t s the fifteenth e x a m p l e of this e n t i t y to b e publiShed a n d the eighth to b e d i a g n o s e d b e f o r e death. C P V A without anomalous pulmonary venous c o n n e c t i o n is u n i f o r m l y fatal, yet if correctly diagn o s e d e a r l y a n d m a n a g e d aggressively m a y b e a m e n a b l e to surgical correction. It is for this r e a s o n that we s u b m i t this report.
from another hospital at seven hours of age. Blood gases on arrival revealed profound acidosis and hypoxemia refractory to treatment; PO2 remained at 19 mm. of Hg. despite oxygen and ventilatory support. On the electrocardiogram there was a right axis at + 150 degrees and marked right ventricular hypertrophy. Chest films (Fig. 1) revealed a normal sized heart. The lungs were hyperinflated and revealed a diffuse reticular pattern consistent with an obstructing lesion distal to the pulmonary capillary bed. On physical examination, the infant was a markedly cyanotic, tachypneic female. The pulse was 160, respiratory rate 80, and systolic blood pressure 60. The lungs were clear. The heart was not enlarged, the second sound was split and accentuated; no murmurs were heard. The heart tones were of poor quality. Cardiac catheterization was performed at ten hours of age. The infant was bradycardiac and cardiac output was difficult to maintain during the procedure. Samples and pressures were obtained in all four chambers and both great vessels. Pulmonary artery pressure was systemic and there was marked right to left shunting. Biplane angiograms were performed from the left ventricle and the main pulmonary artery. The left ventricle, aortic valve and ascending aorta were normal. There was retrograde filling of the ascending aorta on the main pulmonary artery angiogram suggestive of a markedly diminished left ventricular output. The pulmonary artery angiogram (Fig. 2) revealed visualization of a CPV, but no systemic or cardiac connection. A diagnosis of CPVA was made by exclusion of hypoplastic left heart, and total anomalous pulmonary venous return. Following catheterization plans were made for corrective surgery. Her condition continued to deteriorate rapidly, she developed bilateral pneumothorax and had profound uncorrectable acidosis and hypotension and was not considered to be a viable surgical candidate, death occurred at 16 hours of age.
Autopsy Case Report L.S. was a term 2400 gram white female born to a 32 year old gravida 7 para 5 mother. The patient was noted to be cyanotic shortly after birth and was transferred to our special care nursery
1 Present Address: Champlain Valley Hospital, Plattsburg, New York
E x a m i n a t i o n of the h e a r t r e v e a l e d a n atrial septal defect, 5 cm in d i a m e t e r . T h e d u c t u s was p a t e n t . T h e right a n d left p u l m o n a r y veins j o i n e d t o g e t h e r to f o r m a c o m m o n c h a n n e l which did n o t c o m m u n i c a t e with either atrium. T h e azygos s y s t e m was dilated. N o c o m m u n i c a t i o n b e t w e e n the p u l m o n a r y v e n o u s
0301-0449/79/0008/0195/$01.00
196
C.T. DeLise et al.: CommonPulmonaryVein Atresia
Fig. la and b. AP and lateral chest showthat the heart is normal in size. The lungs are over-inflated and reveal a diffuse reticular pattern bilaterally. Fluid is evident in the pleural fissure
Fig. 2. AP angiogram 13 secondsafter the main pulmonary artery injection. Diffuse opacificationof peripheral pulmonary veinspersists and a small CPV can be seen in normal position behind the left atrium ( - ---~).It is not dilated. Faint visualizationof the azygosvein can be appreciated (+ ---~).Additional filmingfor a total of 30 secondsdemonstrated no cardiacchamber opacificationand no other systemic venousvisualization. The opacifiedpulmonaryveins gradually diminished in densityduring the 30 secondillming sequence
system and the heart or systemic veins could be identified. Peribronchial collaterals could not be identified.
Discussion Embryologically, if an obstruction to pulmonary venous return occurs because of failure of incorporation of the CPV into the left atrium, several possible mechanisms to compensate for the defect may be operative depending on the age of the embryo. If obstruction occurs early when primitive systemic venous pathways have not yet regressed, pulmonary venous return may be diverted into one or more of these venous systems. Persistent blood flow through these collateral pathways prevents their normal re-
gression and one of the more c o m m o n types of anomalous pulmonary venous drainage results. If incorporation of the C P V into the left atrium is incomplete and the ostium is stenotic, cor triatriatum is encountered. It has been suggested that C P V A without anomalous pulmonary venous connection results if the failure of incorporation of the C P V into the left atrium occurs somewhat later in embryologic life and the primitive venous pathways have undergone regression. The blood returning from the lungs, therefore, has no well developed path of exodus from the c o m m o n pulmonary vein [1]. The fetus is able to tolerate this severe obstruction to pulmonary venous return because the lungs in utero receive only 8% of the total cardiac output. The little venous return to the CPV can be effectively directed through the peribronchial venous plexuses
C. T. DeLise et al.: Common Pulmonary Vein Atresia
which anastomose with the pulmonary veins to drain the centrolobular and juxtabronchial alveoli [3, 4]. These peribronchial venous plexuses drain into systemic veins at the hilum of the lung and eventually enter the azygos and hemi-azygos systems. Visualization of the azygos vein (Fig. 2) indicates that such collateral pathways were extant in our case. Pulmonary lymphatics may also divert excessive fluid accumulation from the lung in venous obstruction [4, 5]. After birth when the lungs expand, pulmonary blood flow and venous return increase dramatically. The marginal collateral pathways developed in utero are overwhelmed and progressive clinical deterioration results in early death. To date, no infant with CPVA has survived; although successful surgical anastomosis of the CPV to the left atrium has been performed. Extreme acidosis and hypoxia make these infants poor surgical candidates, yet, early diagnosis and surgery under hypothermic arrest remains their only hope for survival.
197
References 1. Lucas, R.V., Jr., Woolfrey, B.V., Anderson, R.C., Lester, R. G., Edwards, J. E.: Atresia of the common pulmonary vein. Pediatrics 29, 729 (1962) 2. Ledbetter, M. K., Wells, D. H., Connors, D. M.: Common pulmonary vein atresia. Am. Heart J. 96, 580 (1978) 3. Roberson, B.: Microangiography of the lung in infancy and childhood. Propius, 1973 4. Liebow, A. A.: The bronchopulmonary venous collateral circulation with special reference to emphysema. Am. J. Pathol. 29,
251 (1953) 5. Rabin, E. R., Meyer, E. C.: Cardiopulmonary effects of pulmonary venous hypertension with special reference to pulmonary lymphatic flow. Circ. Res. 8, 324 (1960) Date of final acceptance: December 13, 1978 Bernard Schneider, M. D. Department of Radiology Crouse-Irving Memorial Hospital 736 Irving Avenue Syracuse, NY 13210 USA
