Stenosis of Pulmonary Veins

Transcription

1 Stenosis of Pulmonary Veins Report of a Patient Corrected Surgically Yasunaru Kawashima, M.D., Takeshi Ueda, M.D., Yasuaki Naito, M.D, Eiji Morikawa, M.D., and Hisao Manabe, M.D. ABSTRACT A 15-year-old boy is reported who underwent surgical correction for stenosis of the right upper and left pulmonary veins at their junction with the left atrium and associated atrial septal defect, ventricular septal defect, and severe pulmonary hypertension. The literature dealing with stenosis of the pulmonary veins is reviewed and the etiology of the lesion and feasibility of preoperative diagnosis are discussed. S tenosis of the pulmonary veins without anomalous pulmonary venous drainage is a rare condition. of the previously reported patients [l-81 had the opportunity to undergo surgical correction of this anomaly; only the theoretical possibility of such an operation was given. A 15-year-old boy was admitted to the Osaka University Hospital in July, He was the product of healthy parents and a normal pregnancy and delivery. He had a history of frequent respiratory infections. At the age of 5 years a cardiac murmur was diagnosed and his activity was restricted. Cyanosis was not apparent during the first ten years of life but was evident thereafter, particularly after exercise. Physical examination revealed a malnourished and slightly cyanotic boy. Clubbing was not evident in his fingers or toes. The lungs were clear to auscultation. A grade 4/6 harsh systolic murmur was heard over the precordium. The pulmonic second sound was split. Blood pressure was 108/60 mm. Hg in the right arm and 128/68 in the leg. The hemoglobin concentration was 14.7 gm. per 100 ml. and the red blood cell count 4.7 million per cubic millimeter. The electrocardiogram showed normal sinus rhythm, right axis deviation to 110 degrees, and a pattern of biventricular hypertrophy. The chest roentgenogram showed a slightly enlarged heart with a prominent main pulmonary artery segment. The pulmonary vasculature appeared to be increased in both proximal lung fields and in the left periphery (Fig. 1). From the First Department of Surgery, Osaka University Medical School, Osaka, Japan. Accepted for publication Aug. 27, Address reprint requests to Dr. Kawashima, First Department of Surgery, Osaka University Hospital, Doojima-hamadoori, Fukushima-ku, Osaka, Japan. 196 THE ANNALS OF THORACIC SURGERY

2 CASE REPORT: Stenosis of Pulmonary Veins FIG. 1. Posteroanterior chest roentgenogram taken before operation shows a prominent main pulmonary artery segment with engorged pulmonary vasculature, particularly in both proximal lung fields and the left periphery. No enlargement of the left atrium is evident. Right heart catheterization (Table 1) showed the pulmonary arterial and systemic arterial pressures to be of the same order. There was evidence of a bidirectional shunt at the atrial level. The pulmonary arterial wedge pressure was not recorded. The diagnosis was made of atrial septal defect with severe pulmonary arterial hypertension. An electrocardiogram and chest roentgenogram showed left ventricular hypertrophy, and an associated ventricular septal defect was suspected. The patient was operated upon July 9, A median sternotomy was performed, and when the pericardium had been opened a distended right upper pulmonary vein was noted. The vein was uncommonly tense, and a thrill was present at the junction with the left atrium. No abnormality of the right lower pulmonary vein was noted. A finger inserted into the right atrium detected the presence of a large atrial septal defect. When the finger was moved forward through the defect into the left side of the atrium, a jet of blood was palpated at the opening of the right upper pulmonary vein. A diagnosis was made of atrial septal defect and stenosis of at least one pulmonary vein at the junction with the left atrium. The patient was heparinized, and cardiopulmonary bypass was established using a disposable plastic sheet oxygenator primed with Ringer's lactate solution. The right atrium was opened widely and an atrial septal defect of ostium secundum type, about 5 cm. long and 3 cm. wide, was noted. The entrance of the right upper pulmonary vein into the left atrium was obstructed by a membranous diaphragm with an opening about 2 mm. in diameter. The right lower pulmonary vein was connected normally with

3 E! n TABLE 1. HEMODYNAMIC DATA BEFORE AND DURING OPERATION Pressure (mm. Hg) Time of Measurement RA RV PA RUPV LA LV Ao Before operation (5) /68 (92)... (5) (96) Before perfusion (3) /33 (51) 33/17 (24) (6) /63 (70) After first perfusion (3) /31 (48) 3117 (16) (13) /57 (77) After second perfusion (7) /12 (21)... (7) /53 (63) RA = right atrium; RV = right ventricle; PA = pulmonary artery; RUPV = right upper pulmonary vein; LA = left atrium; LV = left ventricle; A0 = aorta: ( ) =mean value.

4 CASE REPORT: Stenosis of Pulmonary Veins the left atrium. On the left side the pulmonary veins appeared to unite before reaching the atrium, and at the junction of this common trunk with the atrium a localized thickening narrowed the opening to a diameter of about 5 mm. (Fig. 2). The membranous diaphragm at the junction of the right upper pulmonary vein and the atrium was removed, and the opening of the left common pulmonary vein was widened by incising the thickening at two opposing points. The atrial septal defect was then closed without a patch, the incision in the right atrium was closed, and the patient was taken off cardiopulmonary bypass. The pulmonary artery remained tense, however, and there was a marked thrill in the outflow tract of the right ventricle. The right upper pulmonary venous-left atrial pressure gradient was decreased. The pulmonary arterial pressure, however, remained unchanged, as shown in Table 1. Both dye-dilution and ascorbic acid tests performed for the detection of residual shunt indicated a left-to-right shunt at the ventricular level. The patient was again placed on cardiopulmonary bypass and the right ventricle was opened. A large ventricular septal defect approximately 15 mm. in diameter was noted just beneath the tricuspid valve. This was closed with a Teflon patch. Cardiopulmonary bypass was terminated. There was no longer any thrill in the heart, and the pulmonary arterial pressure had decreased satisfactorily. The patient had an uneventful recovery. Four months after operation he suddenly developed severe hemoptysis that necessitated hospitalization; no further episode of hemoptysis occurred (right upper) (right lower) Membranous PV stenosis - VSD in posterior Tricuspid valve FIG. 2. Schematic drawing of the operative findings. ASD = atrial septal deje6t; PV = pulmonary vein; VSD = Ventricular septal defect. VOL. 12, NO. 8, AUGUST, 1971 igg

5 KAWASHIMA ET AL. FIG. 3. Lung scan taken seven months nfter operation shows little diflerence in uptake between the two lungs and no difference between the upper and lower fields of the right lung. FIG. 4. Photomicrographs of the membranous diaphragm removed from the junction of the right upper pulmonary vein and left atrium, showing intimal fibrous thickening and muscle bundles scattered in the connective tissue. No cell infiltration suggesting active inflammation is left x 4, right x 80, before 40% reduction.) thereafter. Radioisotope scanning of the lung performed seven months after operation indicated slightly diminished blood flow through the left upper lung field but no difference in flow between the upper and lower fields of the right lung (Fig. 3). He continues to do well two years following operation. Histologically, the membranous diaphragm removed from the orifice of the right upper pulmonary vein showed intimal fibrous thickening and scattered muscle bundles surrounded by connective tissue. There was no cell infiltration to suggest active inflammation in the removed diaphragm (Fig. 4). Comment Pulmonary venous stenosis, particularly when not accompanied by anomalous pulmonary venous connection, is rare. In general, both the 200 THE ANNALS OF THORACIC SURGERY

6 CASE REPORT: Stenosis of Pulmonary Veins TABLE 2. PATIENTS WITH PULMONARY VENOUS STENOSIS REPORTED IN THE LITERATURE Patient's Combined Pulmonary Year Age (yr.) Cardiac Vein Reported Source & Sex Malformation ConfiEuration 1951 Reye [73 8, F 1955 Emslie-Smith & associates [51 13, F PDA 1957 Ferencz & Dammann [61 2, Diamond [31 4, M Hamman-Rich syndrome 1959 Bernstein & associates [ll 6, M 1960 Edwards [41 1, M Small ASD Edwards [41 Edwards [41 1, F 10, F Mitral atresia, ASD, VSD Persistent common atrioventricular canal 1962 Shone & associates [81 7 mo., M Large ASD 1967 Contis & associates [21 12, M 1971 Kawashima & associates 15, M ASD, VSD PDA = patent ductus arteriosus; ASD = atrial septal defect; VSD = ventricular septal defect. clinical course and postmortem findings in patients reported previously suggest that the stenosis is congenital rather than acquired. Shone and his co-workers [8] enumerated the factors favoring a congenital basis for this lesion as follows: (1) the frequent association with other congenital anomalies; (2) the young age at which symptoms referable to the lesion become apparent; (3) the similarity of the lesion occurring in normally inserting pulmonary veins to that seen in veins which insert into structures other than the left atrium; and (4) the absence of evidence of active inflammation in or around the involved segment of vein. In addition to these factors, the VOL. 12, NO. 2, AUGUST,

7 KAWASHIMA ET AL. diaphragmatic configuration of the stenotic lesion, the scattered muscle bundles, and the absence of cell infiltration in the diaphragm removed at the time of operation confirm the congenital basis of the lesion in the present patient. Accurate preoperative diagnosis of pulmonary stenosis would seem possible if the stenosis could be visualized by selective angiocardiography. Elevation of pulmonary arterial wedge pressure in the presence of a normal left atrial pressure is suggestive of the lesion. In such an instance, differentiation from cor triatriatum by means of angiocardiography is necessary. No patient in whom an accurate preoperative diagnosis was made has been previously reported, however. In our patient, a bidirectional shunt at the atrial level accompanied by an elevated pulmonary vascular resistance was found at cardiac catheterization. Roentgenographic and electrocardiographic findings, however, showed a hypertrophied left ventricle, suggesting the existence of a ventricular septal defect. Though the rare condition of pulmonary venous stenosis was found at operation, this lesion could not be a cause of left ventricular hypertrophy. In this situation a procedure such as the ascorbic acid test to detect ventricular septal defect should be undertaken prior to perfusion. Patients with stenosis of the pulmonary veins reported previously in the literature are summarized in Table 2. The types of diagrammed stenosis in this table are based on the authors understanding of the reports. There were 4 patients who had no combined cardiac malformation. Two patients had a simple septal defect and 1 patient had patent ductus arteriosus. The feasibility of correcting pulmonary venous stenosis surgically was suggested by Shone and his associates [8] and was carried out in the present patient. References 1. Bernstein, J., Nolke, A. C., and Reed, J. 0. Extrapulmonic stenosis of the pulmonary veins. Circulation 19:891, Contis, G., Fung, R. H., Vawter, G. F., and Nadas, A. S. Stenosis and obstruction of the pulmonary vein associated with pulmonary artery hypertension. Amer. J. Cardiol. 20:718, Diamond, I. The Hamman-Rich syndrome in childhood: Report of a case with unilateral pulmonary arterial and venous stenosis and arteriovenous occlusion. Pediatrics 22:279, Edwards, J. E. Congenital stenosis of pulmonary veins: Pathologic and developmental considerations. Lab. Invest. 9:46, Emslie-Smith, D., Hill, I. G. W., and Low, K. G. Unilateral membranous pulmonary venous occlusion, pulmonary hypertension, and patent ductus. Brit. Heart J. 17:79, Ferencz, C., and Dammann, J. F., Jr. Significance of the pulmonary vascular bed in congenital heart disease: V. Lesion of the left side of the heart causing obstruction of the pulmonary venous return. Circulation 16: 1046, Reye, R. D. K. Congenital stenosis of the pulmonary veins in their extrapulmonary course. Med. J. Aust. 1:801, Shone, J. D., Amplatz, K., Anderson, R. C., Adams, P., Jr., and Edwards, J. E. Congenital stenosis of individual pulmonary veins. Circulation 26:574, THE ANNALS OF THORACIC SURGERY