Catheterization and Cardiovascular Diagnosis 3:67-72
(1977)
Case Reports
SINGLE LEFT PULMONARY VEIN WITH NORMAL PULMONARY VENOUS DRAINAGE: ASSOCIATION WITH PARTIAL ANOMALOUS PULMONARY VENOUS RETURN LCdr P. H. Belott, MC, USNR, Cdr W. V. R. Vieweg, MC, USN, LCdr J. S. Alpert, MC, USN, LCdr V. E. lacovoni, MC, USN, LCdr G. S. Francis, MC, USNR, Allen D. Johnson, M.D., and Capt. A. D. Hagan, MC, USN An unusual case of a single left pulmonary vein draining the lett lung and joining the left atrium without venous obstruction Is reported. This anomaly occurred In assoclation with partial anomalous pulmonary venous return from the upper lobe of the right lung to the superior vena cava. A similar case has been previously reported from our laboratory. The diagnosis of a single left pulmonary vein can be made by routine chest roentgenography because.of the Characteristicradiologic appearance of this anomaly. It Is important to distinguish this benign entity from more ominous pulmonary radlodenslties. Key words: single left pulmonary vein, partial anomalous pulmonary venous return, congenital heart disease
INTRODUCTION
Recently, the characteristic roentgenographic appearance of a single left pulmonary vein with normal pulmonary venous drainage was reported from our laboratory (1). We now report a second case with associated partial anomalous pulmonary venous return from the upper lobe of the right lung. Knowledge of this entity will preclude confusion with more ominous pulmonary radiodensities. Fromthe Naval Regional MedicalCenter, San Diego, California and the University of California at San Dlego School of Medicine The opinions or assertions contained herein are those of the authors and are not to be construed as official or as necessarily reflecting the views of the Medical Department of the iu’avyor the naval service at large. Reprint requests to: CDR W. V. R . Vieweg, MC, USN. Cardiology Division, Naval Regional Medical Center, San Diego, California 92134 Received April 13, 1976; revision accepted August 17, 1976 67
0 1 9 7 7 Alan R. Liss, Inc., 150 F i f t h Avenue, New York, NY 10011
68
Belott et al.
CASE REPORT A 19-year-old serviceman was referred to the Naval Regional Medical Center because of an abnormal chest roentgenogram. The patient was asymptomatic. The physical examination was unremarkable except for a grade I/IV systolic murmur along the left sternal border. Specifically, there was physiologic splitting of the second heart sound. The electrocardiogram demonstrated right ventricular conduction delay. Routine chest roentgenography demonstrated a tortuous snake-like density posterior to the cardiac silhouette. This was best seen in spot films of the heart in the right anterior oblique projection, and tomography in the lateral projection (Fig. 1). Right heart catheterization demonstrated normal pressures including pulmonary arterial wedge pressures measured in both right and left lungs. Mean pulmonary arterial wedge pressures were the same in the right and left lungs; however, phasic pressures in the right lung demonstrated “a” and “v” waves of similar magnitude, but in the left lung the “a” wave measured 5.5 mm Hg and the “v” wave measured 7 mm Hg. Hydrogen inhalation studies demonstrated rapid appearance in the pulmonary trunk, right ventricle, and right atrium. Indocyanine green was selectively injected into each pulmonary artery and sampled in the right atrium. There was early appearance during right pulmonary arterial injection (Fig. 2). Oxygen saturation determinations were 78% in the pulmonary trunk, right ventricle, and right atrium and 70% in the superior vena cava, resulting in a pulmonary to systemic blood flow ratio of 1.5: 1. Highly saturated blood was obtained from the superior vena cava at the site of the pulmonary venous drainage from the upper lobe
Fig. 1A: Spot films of the left ventricle in right anterior oblique projection. Demonstrated is the snakelike appearance of a single left pulmonary vein draining the left lung.
Single Left Pulmonary Vein
69
Fig. 16: On the lateral tomography, the single left pulmonary vein is located behind the heart (arrows).
of the right lung. Pulmonary angiography demonstrated normal pulmonary arterial anatomy. Mainstream and selective injections of the left pulmonary artery demonstrated a tortuous pulmonary venous system entering the left atrium superiorly by way of a single pulmonary vein (Fig. 3A); there was no opacification of the right atrium. Selective injection of the right pulmonary artery revealed an anomalous pulmonary vein draining the upper lobe of the right lung into the superior vena cava (Fig. 3B). On lateral projection (Fig. 3C), mainstream pulmonary angiography demonstrated the single left pulmonary vein posterior to the heart. DISCUSSION Well-described abnormalities of the pulmonary veins include obstruction of pulmonary venous return and various degrees of anomalous pulmonary venous return (2). In 1971, a single left pulmonary vein simulating a pulmonary nodule was first reported (3). In 1974, we reported the characteristic roentgenographic appearance of a single left pulmonary vein (1). A tortuous density in the left chest near the cardiac silhouette is very suggestive of this anomaly.
70
Belott et al.
RPA-RA
18 s e c
Fig. 2: Early appearance of lndocyanine green inthe right atrium following injection into the right pulmonaryartery, in comparison to the normaldye curve obtalnedfollowing injection into the left pulmonary artery. This is consistent with partial anomalous pulmonary venous return from the right lung.
Fig. 3A: Selective left pulmonary arteriogram during levophase demonstrating tortuous pulmo-
nary venous drainage of the left lung entering the left atrium as a single left pulmonary vein in the conventional location of the leflsuperior pulmonaryvein. Notethe absenceof right atrial opacification.
Single Left Pulmonary Vein
71
Fig. 38: Selective right pulmonary arteriogram during levophase demonstrating partial anomalous pulmonary venous return from the upper lobe of the right lung to the superior vena cava (arrow).
In the present case the diagnosis of single left pulmonary vein was suspected prior to cardiac catheterization. At the time of cardiac catheterization, associated partial anomalous pulmonary venous return from the upper lobe of the right lung to the superior vena cava was also found. Normal bilateral pulmonary arterial wedge pressures excluded pulmonary venous obstruction. An atrial septa1 defect was excluded by early appearance of indocyanine green in the right atrium following selective injection in the right pulmonary artery, but not following injection into the left pulmonary artery. In our initial case as well as in the present case, the single left pulmonary vein was physiologically normal. Embryologically the pulmonary vein develops at approximately 29 days from small sprouts of endothelium growing from the superior wall of the left atrium toward the lung bud (4).There is a progressive division into 4 separate pulmonary veins. The presence of a single left pulmonary vein represents a failure of division into superior and inferior left pulmonary veins. In both our cases the single left pulmonary vein drained into the left atrium at the site of the conventional superior left pulmonary vein. Lessons learned from our 2 cases include 1) the characteristic roentgenographic appearance of a single left pulmonary vein draining into the left atrium without obstruction, and 2) the potential association of this anomaly with partial anomalous pulmonary venous return.
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Fig. 3C: Mainstream pulmonary arteriography during levophase on lateral projection demonstrating the single left pulmonary vein posterior to the heart (arrows).
REFERENCES I. Tretheway DG, Francis GS, MacNeii DJ, Vieweg WVR: Single left pulmonary vein with normal pulmonary venous drainage: A roentgenographic curiosity. Am J Cardiol 34:237-239, 1974. 2. Ahmad N , Moller J H , Kanjuh V and Edwards JE: Anomalies of the Pulmonary Veins. Am J Cardiol 20:7769, 1967. 3. Benfield JR, Cots RE, Mills D: Anomalous single left pulmonary vein mimicking a parenchymal nodule. Chest 59:101-103, 1971. 4. Neil1 CA: Development of the pulmonary veins. With reference t o the embryology of anomalies of pulmonary venous return. Pediatrics 18:880487, 1956.
(1977)
Case Reports
SINGLE LEFT PULMONARY VEIN WITH NORMAL PULMONARY VENOUS DRAINAGE: ASSOCIATION WITH PARTIAL ANOMALOUS PULMONARY VENOUS RETURN LCdr P. H. Belott, MC, USNR, Cdr W. V. R. Vieweg, MC, USN, LCdr J. S. Alpert, MC, USN, LCdr V. E. lacovoni, MC, USN, LCdr G. S. Francis, MC, USNR, Allen D. Johnson, M.D., and Capt. A. D. Hagan, MC, USN An unusual case of a single left pulmonary vein draining the lett lung and joining the left atrium without venous obstruction Is reported. This anomaly occurred In assoclation with partial anomalous pulmonary venous return from the upper lobe of the right lung to the superior vena cava. A similar case has been previously reported from our laboratory. The diagnosis of a single left pulmonary vein can be made by routine chest roentgenography because.of the Characteristicradiologic appearance of this anomaly. It Is important to distinguish this benign entity from more ominous pulmonary radlodenslties. Key words: single left pulmonary vein, partial anomalous pulmonary venous return, congenital heart disease
INTRODUCTION
Recently, the characteristic roentgenographic appearance of a single left pulmonary vein with normal pulmonary venous drainage was reported from our laboratory (1). We now report a second case with associated partial anomalous pulmonary venous return from the upper lobe of the right lung. Knowledge of this entity will preclude confusion with more ominous pulmonary radiodensities. Fromthe Naval Regional MedicalCenter, San Diego, California and the University of California at San Dlego School of Medicine The opinions or assertions contained herein are those of the authors and are not to be construed as official or as necessarily reflecting the views of the Medical Department of the iu’avyor the naval service at large. Reprint requests to: CDR W. V. R . Vieweg, MC, USN. Cardiology Division, Naval Regional Medical Center, San Diego, California 92134 Received April 13, 1976; revision accepted August 17, 1976 67
0 1 9 7 7 Alan R. Liss, Inc., 150 F i f t h Avenue, New York, NY 10011
68
Belott et al.
CASE REPORT A 19-year-old serviceman was referred to the Naval Regional Medical Center because of an abnormal chest roentgenogram. The patient was asymptomatic. The physical examination was unremarkable except for a grade I/IV systolic murmur along the left sternal border. Specifically, there was physiologic splitting of the second heart sound. The electrocardiogram demonstrated right ventricular conduction delay. Routine chest roentgenography demonstrated a tortuous snake-like density posterior to the cardiac silhouette. This was best seen in spot films of the heart in the right anterior oblique projection, and tomography in the lateral projection (Fig. 1). Right heart catheterization demonstrated normal pressures including pulmonary arterial wedge pressures measured in both right and left lungs. Mean pulmonary arterial wedge pressures were the same in the right and left lungs; however, phasic pressures in the right lung demonstrated “a” and “v” waves of similar magnitude, but in the left lung the “a” wave measured 5.5 mm Hg and the “v” wave measured 7 mm Hg. Hydrogen inhalation studies demonstrated rapid appearance in the pulmonary trunk, right ventricle, and right atrium. Indocyanine green was selectively injected into each pulmonary artery and sampled in the right atrium. There was early appearance during right pulmonary arterial injection (Fig. 2). Oxygen saturation determinations were 78% in the pulmonary trunk, right ventricle, and right atrium and 70% in the superior vena cava, resulting in a pulmonary to systemic blood flow ratio of 1.5: 1. Highly saturated blood was obtained from the superior vena cava at the site of the pulmonary venous drainage from the upper lobe
Fig. 1A: Spot films of the left ventricle in right anterior oblique projection. Demonstrated is the snakelike appearance of a single left pulmonary vein draining the left lung.
Single Left Pulmonary Vein
69
Fig. 16: On the lateral tomography, the single left pulmonary vein is located behind the heart (arrows).
of the right lung. Pulmonary angiography demonstrated normal pulmonary arterial anatomy. Mainstream and selective injections of the left pulmonary artery demonstrated a tortuous pulmonary venous system entering the left atrium superiorly by way of a single pulmonary vein (Fig. 3A); there was no opacification of the right atrium. Selective injection of the right pulmonary artery revealed an anomalous pulmonary vein draining the upper lobe of the right lung into the superior vena cava (Fig. 3B). On lateral projection (Fig. 3C), mainstream pulmonary angiography demonstrated the single left pulmonary vein posterior to the heart. DISCUSSION Well-described abnormalities of the pulmonary veins include obstruction of pulmonary venous return and various degrees of anomalous pulmonary venous return (2). In 1971, a single left pulmonary vein simulating a pulmonary nodule was first reported (3). In 1974, we reported the characteristic roentgenographic appearance of a single left pulmonary vein (1). A tortuous density in the left chest near the cardiac silhouette is very suggestive of this anomaly.
70
Belott et al.
RPA-RA
18 s e c
Fig. 2: Early appearance of lndocyanine green inthe right atrium following injection into the right pulmonaryartery, in comparison to the normaldye curve obtalnedfollowing injection into the left pulmonary artery. This is consistent with partial anomalous pulmonary venous return from the right lung.
Fig. 3A: Selective left pulmonary arteriogram during levophase demonstrating tortuous pulmo-
nary venous drainage of the left lung entering the left atrium as a single left pulmonary vein in the conventional location of the leflsuperior pulmonaryvein. Notethe absenceof right atrial opacification.
Single Left Pulmonary Vein
71
Fig. 38: Selective right pulmonary arteriogram during levophase demonstrating partial anomalous pulmonary venous return from the upper lobe of the right lung to the superior vena cava (arrow).
In the present case the diagnosis of single left pulmonary vein was suspected prior to cardiac catheterization. At the time of cardiac catheterization, associated partial anomalous pulmonary venous return from the upper lobe of the right lung to the superior vena cava was also found. Normal bilateral pulmonary arterial wedge pressures excluded pulmonary venous obstruction. An atrial septa1 defect was excluded by early appearance of indocyanine green in the right atrium following selective injection in the right pulmonary artery, but not following injection into the left pulmonary artery. In our initial case as well as in the present case, the single left pulmonary vein was physiologically normal. Embryologically the pulmonary vein develops at approximately 29 days from small sprouts of endothelium growing from the superior wall of the left atrium toward the lung bud (4).There is a progressive division into 4 separate pulmonary veins. The presence of a single left pulmonary vein represents a failure of division into superior and inferior left pulmonary veins. In both our cases the single left pulmonary vein drained into the left atrium at the site of the conventional superior left pulmonary vein. Lessons learned from our 2 cases include 1) the characteristic roentgenographic appearance of a single left pulmonary vein draining into the left atrium without obstruction, and 2) the potential association of this anomaly with partial anomalous pulmonary venous return.
72
Belott et al.
Fig. 3C: Mainstream pulmonary arteriography during levophase on lateral projection demonstrating the single left pulmonary vein posterior to the heart (arrows).
REFERENCES I. Tretheway DG, Francis GS, MacNeii DJ, Vieweg WVR: Single left pulmonary vein with normal pulmonary venous drainage: A roentgenographic curiosity. Am J Cardiol 34:237-239, 1974. 2. Ahmad N , Moller J H , Kanjuh V and Edwards JE: Anomalies of the Pulmonary Veins. Am J Cardiol 20:7769, 1967. 3. Benfield JR, Cots RE, Mills D: Anomalous single left pulmonary vein mimicking a parenchymal nodule. Chest 59:101-103, 1971. 4. Neil1 CA: Development of the pulmonary veins. With reference t o the embryology of anomalies of pulmonary venous return. Pediatrics 18:880487, 1956.
