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Histology of Pulmonary Arterial Supply in Pulmonary Atresia with Ventricular Septal Defect GAETANO THIENE, M.D., CARLA FRESCURA, M.D., ROBERTA M. BINI, M.D. MARIALUISA VALENTE, M.D., AND VINCENZO GALLUCCI, M.D. Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 SUMMARY A histologic study was performed on 22 specimens of pulmonary atresia with ventricular septal defect to 1) ascertain the existence of the main pulmonary artery; 2) distinguish the ductus arteriosus from the systemic collateral arteries (SCA); 3) establish the nutritive or functional nature of collateral circulation; and 4) evaluate the morphology of the distal pulmonary bed. Three cases had absent main pulmonary artery, one with and two without signs of infundibular septation. We suggest that absent main pulmonary artery may exist with both infundibular pulmonary atresia and persistent truncus arteriosus. SCAs have been found to have similar histological features as systemic muscular arteries of the same size their medial muscular layer merges gradually into an elastic one at different depth inside the lungs. Injection of contrast material allowed us to demonstrate that these vessels are functional, since they inosculate into efficient pulmonary arteries ending in the respiratory units. When the distal pulmonary vascular bed is perfused by large SCAs, proliferative lesions like those found in large left-to-right shunts may occur. Early in infancy, banding of large, nonstenotic SCAs could protect the distal pulmonary vasculature. Moreover, total surgical repair should be associated with ligation of the SCA to avoid residual left-to-right shunt, if the pulmonary arteries can carry the full pulmonary blood flow. A HISTOLOGIC STUDY of the pulmonary arterial supply in pulmonary atresia with ventricular septal defect (VSD) is important for several reasons. First, the macroscopic dissection cannot always reveal the existence of a pulmonary trunk arising from the heart.' This is essential in distinguishing cases without from cases with atresia of the main pulmonary artery, and may suggest whether they represent embryologically a form of persistent truncus arteriosus or a pulmonary atresia with VSD.2 Second, only by histology it is possible to recognize whether a collateral branch that arises from the aortic arch is a ductus arteriosus or a systemic collateral artery.8-10 Third, debate exists on whether the collateral arterial supply should be considered functional or nutritive. These vessels are generally reported as a compensatory dilatation or hypertrophy of bronchial arteries."-14 Stenosis of the collateral vessels may be visualized by angiocardiography,"-21 but their morphologic substrate is unknown. Finally, some patients with pulmonary atresia actually have an increased pulmonary blood flow and eventually become cyanotic.'2, 16, 22 The question arises whether pulmonary vascular disease occurs in this congenital malformation as much as in other defects with left-toright shunt. Definition of Terms Pulmonary atresia with VSD is the malformation in which both ventricles eject only into the aorta. A pulmonary trunk usually arises from the cardiac base at least as a fibrous stem. The pulmonary circulation is supplied by a patent ductus arteriosus or by systemic collateral arteries originating from the descending aorta. Truncus type IV is also a cardiac anomaly in which the pulmonary arterial supply comes through systemic collateral arteries or a ductus arteriosus, but in which neither a main pulmonary artery nor an infundibular septum is identifiable. Material and Methods Twenty-two hearts were selected from our anatomic collection according to the above mentioned definitions. All specimens had visceroatrial situs solitus, levocardia and atrioventricular concordance. The great arteries were normally related when the pulmonary trunk was identifiable. Cases with posterior atretic pulmonary trunk (so-called transposition with pulmonary atresia and VSD)24 have been excluded from our study. All patients died within the first year of life. According to the pattern of pulmonary arterial supply, the specimens were divided as follows: 11 cases in which the pulmonary arterial supply was solely ductus-dependent; seven cases without ductus arteriosus in which the pulmonary arterial circulation depended on small systemic collateral arteries arising from the descending aorta; three cases without ductus arteriosus and with large systemic collateral arteries; and one case with a left ductus arteriosus that supplied the left lung and two large systemic collateral arteries that supplied the right lung. From the Departments of Pathology, Cardiology, Pediatrics and Cardiovascular Surgery, University of Padova Medical School, Padova, Italy. Supported in part by contract 78.02306.05, Consiglio Nazionale delle Ricerche, Rome, Italy. Address for correspondence: Dr. Gaetano Thiene, Department of Pathology, Via Gabelli 61, 35100 Padova, Italy. Received January 30, 1979; revision accepted April 30, 1979. Circulation 60, No. 5, 1979. 1066 PULMONARY ATRESIA WITH VSD/Thiene et al. 1 067 Segments from the systemic collateral arteries and the ductus arteriosus and blocks from multiple areas of the lungs were embedded in paraffin, sectioned 7y thick and stained with hematoxylin and eosin and elastic Van Gieson. The great vessels at the cardiac base were examined histologically when the pulmonary trunk was not seen on gross examination. In three patients, postmortem angiography of the pulmonary arterial supply was performed before fixation and histologic examination. pulmonary arteries; the intracardiac anatomy had an extremely deviated infundibular septum. The fourth heart showed a similar extracardiac anatomy, but we did not see an infundibular septum in the right ventricular outflow tract. On histologic examination the presence of a vessel with an elastic media was demonstrated only in the last case. This vessel, which lay on the left side of the aortic root, was the atretic pulmonary trunk (fig. 1). Results Histology of the Ductus Arteriosus and the Systemic Collateral Arteries The ductus arteriosus had the features of a closing Histology of the Great Vessels at the Cardiac Base Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 In four specimens a fibrous strand arising from the heart base as a vestige of the pulmonary trunk could not be positively identified by gross examination. In two of these, derivatives of the sixth aortic arches, i.e., ductus arteriosus and pulmonary arteries, were not observed either inside the pericardial cavity or at the pulmonary hila. The pulmonary arterial circulation in these cases was supplied by systemic arteries that arose from the descending aorta; the intracardiac anatomy did not show any signs of infundibular septation. The third heart had a right aortic arch and a left ductus arteriosus connected with two confluent ductus with intimal proliferation, fragmented internal elastic lamina, mucoid lakes and cytolitic necrosis of the media, which appeared musculoelastic.10 The systemic collateral arteries had a muscular media with absent external elastic lamina and a thick adventitia, like any other systemic artery of the same diameter. The media merged gradually into a thin elastic layer; large systemic arteries lost their muscular features near the hilum, while small systemic arteries maintained their muscularity up to the periphery of the lungs (fig. 2). Sections from stenotic segments of systemic collateral arteries disclosed focal fibrous intimal thickenings (fig. 3). N /&S 1. t .4 .. FIGURE 1. Histology of the root of the great arteries in a case with apparent absence of the main pulmonary artery. A small vascular structure with elastic medial layer is visible aside the aorta. A = aorta; P = pulmonary artery. Elastic-Van Gieson stain; original magnification A) X 12, B) X 48. l1068 CIRCULATION Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 WIaI , I v I I" 39 - I T < * * |~1 23 14CHisI -v..r c I, 6-"''D VOL 60, No 5, NOVEMBER 1979 PULMONARY ATRESIA WITH VSD/Thiene et al. 1069 Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 FIGURE 3. Pulmonary atresia with ventricular septal defect: segments of large collateral arteries with fibrous intimal proliferations (A and B) and rupture of the internal elastic lamina (A). Elastic-Van Gieson stain; original magnification X 60. Histology of the Lungs Histologic examination of the lungs was performed in 15 cases. Figure 4 shows the degree of the pulmonary vascular disease25 plotted against age, according to the pattern of pulmonary arterial supply. Medial hypertrophy, intimal proliferation and even plexiform lesions were observed only in pulmonary segments perfused by large, nonstenotic systemic collateral artries (fig. 5). Progressive thinning of the media of the small pulmonary arteries and arterioles occurred with age only in those cases with pulmonary arterial supply that depended on a ductus arteriosus and/or small or stenotic systemic collateral arteries. In one patient with multifocal pulmonary arterial supply, different degrees of pulmonary vascular disease were detected. Serial sections, including collaterals and their lung segments, showed that the contrast material injected postmortem passed from systemic arteries with muscular media, through elastic FIGURE 2. Pulmonary atresia with ventricular septal defect and pulmonary circulation supplied by collateral arteries. A large systemic-pulmonary artery together with its origin from the aorta (A) and its branching inside the lung has been dissected and serially cut histologically. The systemic muscular media (B) merges gradually into a pulmonary elastic layer (C and D). ElasticVan Gieson stain; original magnification B) X 48, C and D) X 190. CIRCULATION 1070 PULMONARY ATRESIA AND VENTRICULAR SEPTAL DEFECT PULMONARY VASCULAR DISEASE IN 15 PATIENTS 12 . . 9 in ~6- VOL 60, No 5, NOVEMBER 1979 histologic examination to have a small elastic vessel next to the aortic root. Among the remaining three, two had an intracardiac anatomy typical of truncus arteriosus and one showed an anteriorly deviated infundibular septum that completely obstructed the intracardiac pulmonary outflow as seen in typical pulmonary atresia with VSD. A Nature of the Collateral Circulation o 5z , 4E 32- A 0 A 0 *A AAA 1- O 2 1 3 GRAOE OF PULMONARY VASCULAR DISEASE 4 Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 ARTERIAL SUPPLY DEPENDING BY:A oUCTUS ARTERIOSUS * SMALL OR STENOTIC ARTERIES * LARGE ARTERIES WITHOUT STENOSIS * SAME PATIENT IN DIFFERENT AREAS FIGURE 4. Pulmonary atresia with ventricular septal defect. Grades of pulmonary vascular disease according to the Heath and Edwards classification25 are plotted against age in 15 patients with reference to the pattern ofpulmonary arterial supply. arteries, to small arteries, arterioles and capillaries of the respiratory units (fig. 6). Discussion Absence of the Main Pulmonary Artery: A Form of Persistent Truncus Arteriosus or Pulmonary Atresia with VSD? Many cardiovascular anomalies cannot be considered either a classic truncus arteriosus communis or a truncus solitarius aorticus or pulmonalis. Whether the single trunk is a persistent truncus or a normal aorta is controversial. This malformation has been considered a pulmonary atresia with VSD16, 17, 19, 26 or a solitary aortic trunk with agenesis of the pulmonary arteries,3 8 or as a persistent ventral aorta with agenesis of both the sixth aortic arches and the aorticopulmonary septum.27 Manhoff and Howe28 first recognized the absence of the main pulmonary artery as a separate nosographic entity and described two main forms: 1) single trunk with lungs supplied by arteries arising from the descending aorta or by other anomalous arteries; 2) single trunk with lungs supplied by ductus arteriosus. The first arterial pattern was classified by Collett and Edwards as persistent truncus arteriosus type IV,23 while the latter was described by Edwards' group as ductal origin of the pulmonary arteries.29 30 We believe that both intraand extracardiac features are essential in distinguishing these two anomalies. In fact, absence of the main pulmonary artery may be present in pulmonary infundibular atresia (pseudotruncus) as well as in persistent truncus arteriosus (truncus type IV). Among our four cases with absent pulmonary trunk on gross examination, only one was found on It is generally believed that the development of the collateral circulation takes place after birth, due to hypertrophy and dilatation of the nutritive circulation of the bronchial arteries, while the ductus arteriosus undergoes its natural closure. However, in 1947 Taussig observed that in pulmonary atresia with VSD the ductus arteriosus may often be absent.31 Bharati et al. confirmed this observation in autopsy material.32 We observed collateral arteries even in newborns, suggesting that collateral circulation must be present before birth and may represent the persistence of the primitive arterial connections between the aorta and the pulmonary plexus.6 Absence of the ductus arteriosus has been described in persistent truncus arteriosus type I and II,4 in tetralogy of Fallot with absent pulmonary valve,34 in dogs with pulmonary atresia and VSD,35 and it has been illustrated by Van Mierop et al. in a dog embryo with persistent truncus arteriosus.7 These collateral arteries have the same histologic features as any other systemic muscular artery of the same size. Distally the muscular media merges gradually, at different depth inside the lungs, into an elastic media. The injection of the contrast material allowed us to visualize these vessels inosculated into efficient pulmonary arteries, ending at the respiratory units where gas exchange occurs. Since this circulation is functional and not nutritive,36 the term "bronchial arteries" is inaccurate to describe the collateral vessels. Pulmonary Vascular Disease A histologic study of the lungs in pulmonary atresia has rarely been performed and no special attention has been paid so far to the pattern of the pulmonary circulation or to the difference between cases with or without VSD.9 7 Despite the complete obstruction of the pulmonary outflow, large systemic collateral arteries may be responsible for increased pulmonary blood flow. In these instances, the pulmonary arterial vascular bed may have pathologic features similar to those in large left-to-right shunts." 25 Medial hypertrophy, intimal proliferations and even plexiform lesions were observed in our cases. In such patients, cyanosis may, therefore, worsen as a consequence of pulmonary vascular disease. We did not perform a quantitative analysis of structural pulmonary features, although such an investigation would be of value also in pulmonary atresia with VSD. There is evidence that the high pulmonary vascular resistance may be due to insufficient develop- PULMONARY ATRESIA WITH VSD/Thiene et al. 1071 Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 FIGURE 5. Pulmonary alresia with ventricular septal defect. obstructive pulmonary vascular disease with medial hypertrophy, intimal proliferation (A) and plexiform lesions (B) in pulmonary segments perfused by large, unobstructed collateral arteries. Hematoxylin and eosin stain; original magnification A) X 190, B) X 120. ment of the intra-acinar pulmonary circulation in addition to the hypertensive vascular disease.40 Surgical Considerations Our histologic findings show that the peripheral vessels function for gas exchange, regardless of the source of blood supply. If repair is to be attempted, true pulmonary arteries must be demonstrated.15-2, 41, 42, 45 Surgical reconstruction can be performed when at least one well-developed true central pulmonary artery can be identified at the pulmonary hila.'14 41 Both central pulmonary arteries were absent in only two of our cases. A previously reported gross examination of our specimen6 disclosed that in 40% of the cases the pulmonary arteries were too diminutive to be used for total repair. Progressive enlargement of hypoplastic pulmonary arteries may be obtained surgically by reconstructing right ventricle-to-pulmonary artery continuity with a pericardial patch graft, leaving the VSD unrepaired.43 If no stenoses occur in the systemic collateral arteries before the peripheral distribution, the ultimate pathologic changes will result in severe pulmonary vascular disease. Banding of large, nonstenotic collateral vessels could be indicated early in infancy to protect the lungs. Nevertheless, the obliterative lesions may be unequally distributed, depending on the pattern of pulmonary arterial supply. In one patient in whom the left lung was perfused entirely by the ductus arteriosus and the right lung by Voi 60, No 5, NOVEMBER 1979 CIRCULATION 1072 Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 PULMONARY ATRESIA WITH VSD/Thiene et al. 1073 FIGURE 6. Pulmonary atresia with ventricular septal defect and large collateral arteries. Contrast material injected into a large collateral artery opacifies part of the superior lobe of the right lung, with regular peripheral ramification (A). Histologic sections reveal contrast material inside large elastic arteries (B), musculoelastic arteries (C), small muscular arteries with intimal proliferation, and inside the alveolar capillaries of the respiratory units (D). Elastic-Van Gieson stain; original magnification B) X 60, C and D X 190. two large systemic collateral arteries, the pulmonary vascular disease was confined in the right lung; the whole left lung was spared and could be used for surgical reconstruction. This case suggests that very accurate hemodynamic assessment is mandatory before making a decision of inoperability. To avoid residual left-to-right shunt, total surgical repair should be associated with ligation of the collateral vessels if the pulmonary arteries are capable of carrying the full pulmonary blood flow.'4 References 19. 20. 21. Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 1. Sotomora RF, Edwards JE: Anatomic identification of socalled absent pulmonary artery. Circulation 57: 624, 1978 2. Lampertico P: Persistent truncus arteriosus comunis. Folia 22. Hered Path (suppl IV), Sapil, Milan, 1964 3. Van Praagh R, Van Praagh S: The anatomy of common aortico-pulmonary trunk (truncus arteriosus comunis) and its embryologic implications. A study of 57 necropsy cases. Am J Cardiol 16: 406, 1965 4. Thiene G, Bortolotti U, Gallucci V, Terribile V, Pellegrino PA: Anatomical study of truncus arteriosus comunis with embryological and surgical considerations. Br Heart J 38: 1109, 1976 5. Crupi G, Macartney FJ, Anderson RH: Persistent truncus arteriosus. A study of 66 autopsy cases with special reference to definition and morphogenesis. Am J Cardiol 40: 569, 1977 6. Thiene G, Bortolotti U, Gallucci V, Valente ML, Dalla Volta S: Pulmonary atresia with ventricular septal defect. Br Heart J 39: 1223, 1977 7. Van Mierop LHS, Patterson DF, Schnarr WR: Pathogenesis of persistent truncus arteriosus in light of observations made in dog embryo with the anomaly. Am J Cardiol 41: 755, 1978 8. Stone FM, Amplatz K, Lucas RV, Fukida T, Edwards JE: Ventricular septal defect, solitary aortic trunk and ductal origin of pulmonary arteries. Am Heart J 92: 506, 1976 9. Van Praagh R, Ando M, Van Praagh S, Senno A, Hougen TJ, Novak G, Hastreiter AR: Pulmonary atresia: anatomic considerations. In The Child with Congenital Heart Disease after Surgery, edited by Langford and Rowe, New York, 1976 10. Gittemberger De Groot AC: Persistent ductus arteriosus: most probably a primary congenital malformation. Br Heart J 39: 610, 1977 11. Wagenvoort CA, Heath D, Edwards JE: The pathology of the pulmonary vasculature. Springfield, Ill, Charles C Thomas, 1964 12. Stuckey D, Bowdler JD, Reye RDK: Absent sixth aortic arch: a form of pulmonary atresia. Br Heart J 30: 258, 1968 13. Edwards JE, McGoon DC: Absence of anatomic origin from the heart of pulmonary arterial supply. Circulation 47: 393, 23. 1973 14. McGoon DC, Baird DK, Davis GD: Surgical management of large bronchial collateral arteries with pulmonary stenosis or atresia. Circulation 52: 109, 1975 15. Chesler E, Beck W, Schrire V: Selective catheterization of pulmonary or bronchial arteries in the preoperative assessment of pseudotruncus arteriosus and truncus arteriosus type IV. Am J Cardiol 26: 20, 1970 16. Jefferson K, Rees S, Somerville J: Systemic arterial supply to the lungs in pulmonary atresia and its relation to pulmonary artery development. Br Heart J 34: 418, 1972 17. Macartney FJ, Deverall P, Scott 0: Haemodynamic characteristics of systemic arterial blood supply to the lungs. Br Heart J 35: 28, 1973 R, Beck W: The assessment of the arterial supply to the lungs in pseudotruncus arteriosus and truncus arteriosus type IV in relation to surgical repair. Am Heart J 88: 542, 1974 Macartney FJ, Scott 0, Deverall P: Haemodynamic and 18. Chesler E, Matisonn 24. 25. anatomical characteristics of pulmonary blood supply in pulmonary atresia with ventricular septal defect including a case of persistent fifth aortic arch. Br Heart J 36: 1049, 1974 McGoon MD, Fulton RF, Davis GD, Ritter DG, Neill CA, White RI Jr: Systemic collateral and pulmonary artery stenosis in patients with congenital pulmonary valve atresia and ventricular septal defect. Circulation 56: 473, 1977 Soto B, Pacifico AD, Luna RF, Bargeron LM Jr: A radiographic study of congenital pulmonary atresia with ventricular septal defect. Am J Roentgenol 129: 1027, 1977 Danilowicz D, Ross J Jr: Pulmonary atresia without cyanosis. Report of two cases with ventricular septal defect and increased pulmonary blood flow. Br Heart J 33: 138, 1971 Collett RW, Edwards JE: Persistent truncus arteriosus: a classification according to anatomic types. S Clin North Am 29: 1245, 1949 Marcelletti C, Mair DC, McGoon DC, Wallace RB, Danielson GK: Complete repair of transposition of the great arteries with pulmonary atresia. J Thorac Cardiovasc Surg 72: 215, 1976 Heath D, Edwards JE: The pathology of hypertensive pulmonary vascular disease. A description of six grades with special reference to congenital cardiac septal defects. Circula- tion 18: 533, 1958 26. Somerville J: Management of pulmonary atresia. Br Heart J 32: 641, 1970 27. Angelini P, Leachman RD: Trunco-conal septal defects. An anatomic and embryologic discussion of common truncus and related malformations. Eur J Cardiol 2: 11, 1974 28. Manhoff LJ, Howe JS: Absence of the pulmonary artery: a new classification for pulmonary arteries of anomalous origin. Arch Pathol 48: 155, 1949 29. Murray CA, Korns ME, Amplatz K, Edwards JE: Bilateral origin of pulmonary artery from homolateral ductus arteriosus. Chest 58: 310, 1970 30. Todd EP, Lindsay WG, Edwards JE: Bilateral ductal origin of the pulmonary arteries. Systemic-pulmonary arterial anastomosis as first stage in planned total correction. Circulation 54: 834, 1976 31. Taussig HB: Clinical and pathological findings in cases of truncus arteriosus in infancy. Am J Med 2: 26, 1947 32. Bharati S, Paul MH, Idriss FS, Potkin RT, Lev M: The surgical anatomy of pulmonary atresia with ventricular septal defect. Pseudotruncus. J Thorac Cardiovasc Surg 69: 713, 1975 33. Bharati S5 McAllister HA Jr, Rosenquist GC, Miller RA, Tatooles CJ, Lev M: The surgical anatomy of truncus arteriosus comunis. J Thorac Cardiovasc Surg 68: 501, 1974 34. Emmanouilides GC, Thanopoulos B, Siassi B, Fishbein M: Agenesis of ductus arteriosus associated with the syndrome of tetralogy of Fallot and absent pulmonary valve. Am J Cardiol 37: 403, 1976 35. Patterson DF, Pyle RD, Van Mierop LHS, Melbin J, Olson M: Hereditary defects of the conotruncal septum in Keeshond dogs: pathologic and genetic studies. Am J Cardiol 34: 183, 1974 36. Daliento L, Stritoni P, Chioin R, Frescura C, Thiene G: Systemic-pulmonary arterial supply in pulmonary atresia and ventricular septal defect. Postmortem angiography and histological survey in a case. Chest 74: 685, 1978 37. Wagenvoort CA, Edwards JE: The pulmonary arterial tree in pulmonic atresia. Arch Pathol 71: 56, 1961 38. Ferencz C: The pulmonary vascular bed in tetralogy of Fallot. Bull J Hopkins Hosp 106: 81, 1960 angiography. Circulation 58: 140, 1978 43. Gill CC, Moodie DS, McGoon DC: Staged surgical management of pulmonary atresia with diminutive pulmonary arteries. J Thorac Cardiovasc Surg 73: 436, 1977 44. Kirklin JW, Bargeron LM Jr, Pacifico AD: The enlargement of small pulmonary arteries by preliminary palliative operations. Circulation 56: 612, 1977 45. Singh S: Demonstration of pulmonary arteries by contrast injection into a pulmonary vein. Presented at the 15th Annual General Meeting of the Association of European Paediatric Cardiologists, Gent, Belgium, 1977 39. Haworth SG, Reid L: Quantitative structural study of pulmonary circulation in the newborn with pulmonary atresia. Thorax 32: 129, 1977 40. Haworth SG, Sauer U, Buhlmeyer C, Reid L: Development of the pulmonary circulation in ventricular septal defect: a quantitative structural study. Am J Cardiol 40: 781, 1977 41. Davis GD, Fulton RE, Ritter DG, Mair DD, McGoon DC: Congenital pulmonary atresia with ventricular septal defect: angiographic and surgical correlates. Radiol 128: 133, 1978 42. Nihill MR, Mullins CE, McNamara DG: Visualization of the pulmonary arteries in pseudotruncus by pulmonary vein wedge Effects of Acute Hemodynamic Alterations on Pulmonic Valve Motion Experimental and Clinical Echocardiographic Studies Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 RICHARD E. KERBER, M.D., JAMES B. MARTINS, M.D., ROBERT BARNES, M.D., WALTER J. MANUEL, AND MICHAEL MAXIMOV, M.D. SUMMARY The purpose of this study was to assess the effects of acute alterations of the pulmonary circulation on the pulmonic valve echocardiogram. We measured the pulmonic valve opening velocity (PVOV) and right-sided systolic time intervals (right ventricular preejection period-to-right ventricular ejection time ratio [RPEP/RVET] ) in 22 open-chest dogs subjected to acute hemodynamic alterations produced by inferior vena cava constriction, atrial pacing, isoproterenol infusion and microsphere embolization of the pulmonary artery. We found only fair correlations between PVOV and peak pulmonary artery flow (r 0.56), right ventricular dp/dt (r = 0.43), stroke volume (r = 0.42), pulmonary artery systolic pressure (r = 0.33) and peak pulmonary artery acceleration (r = 0.31). RVET was shortened by reduced venous return (caval constriction) and by increases in heart rate (atrial pacing and isoproterenol), which resulted in increases in RPEP/RVET that did not correspond well to simultaneous changes in pulmonary artery pressure. In seven patients breathing 10% 02 to raise pulmonary artery pressure acutely, we found no change or a fall in PVOV. Thus, the pulmonic valve echocardiogram is influenced by multiple factors relating to parameters of pulmonary flow and right ventricular contractility, and may be of limited clinical usefulness in predicting pulmonary artery pressures. = CHARACTERISTIC ALTERATIONS of echocardiographic pulmonic valve motion in the presence of pulmonary hypertension have been reported.' These include rapid pulmonic valve opening velocity (PVOV),' attenuation or abolition of the normal presystolic pulmonic valve opening ("a" dip),'. 2 and prolongation of the right ventricular preejection period-to-right ventricular ejection time ratio (RPEP/ RVET).3 4 Several authors have suggested, on the basis of these studies, that pulmonic valve echocardiography may be useful in the serial assessment of the pulmonary circulation and permit noninvasive detection of developing pulmonary hypertension.1' 4 However, all the studies reported have been performed on patients with chronic pulmonary hypertension. Little information is available on the effects of acute changes in pulmonary artery pressure on pulmonic valve motion. In fact, the determinants of pulmonic valve motion in general are not known. Therefore, to evaluate the effects of various acute hemodynamic alterations on the pulmonic valve echocardiogram and to determine what factors influence pulmonic valve motion, we performed experiments in both animals and patients. Methods Animal Studies Twenty-two dogs were anesthetized with intravenous chloralose-urethane, intubated and ventilated with a Harvard respirator. A mid-sternal thoracotomy was performed. The pericardium was incised anteriorly and sutured to the chest wall so that the heart was suspended in a pericardial cradle. A #7F Swan-Ganz catheter was inserted into the right ventri- From the Departments of Internal Medicine and Thoracic Surgery, The University of Iowa Hospital, Iowa City, Iowa. Supported in part by NHLBI grant HL-014388, and Iowa Heart Association grant 76-G-16. Address for correspondence: Richard E. Kerber, M.D., Department of Medicine, University of Iowa Hospital, Iowa City, Iowa 52242. Received February 2, 1979; revision accepted April 17, 1979. Circulation 60, No. 5, 1979. 1074 Histology of pulmonary arterial supply in pulmonary atresia with ventricular septal defect. G Thiene, C Frescura, R M Bini, M Valente and V Gallucci Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 Circulation. 1979;60:1066-1074 doi: 10.1161/01.CIR.60.5.1066 Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Copyright © 1979 American Heart Association, Inc. All rights reserved. Print ISSN: 0009-7322. Online ISSN: 1524-4539 The online version of this article, along with updated information and services, is located on the World Wide Web at: http://circ.ahajournals.org/content/60/5/1066 Permissions: Requests for permissions to reproduce figures, tables, or portions of articles originally published in Circulation can be obtained via RightsLink, a service of the Copyright Clearance Center, not the Editorial Office. 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