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Pulsed Wave and Color Doppler Echocardiography and Cardiac Catheterization Findings in Bilateral Pulmonary Vein Stenosis
Pulsed Wave and Color Doppler Echocardiography and Cardiac Catheterization Findings in Bilateral Pulmonary Vein Stenosis Jong-Won Ha, MD, Namsik Chung, MD, Junghan Yoon, MD, Yangsoo Jang, MD, Byung-Ok Kim, MD, Seung-Yun Cho, MD, and Bum-Koo Cho, MD, Seoul, Korea
Pulmonary vein stenosis is a rare condition that usually is congenital in origin; it is almost invariably fatal in its bilateral and severe forms. It is often overlooked, however, during clinical examination, routine echocardiography, and even at cardiac catheterization. This report
P
ulmonary vein stenosis is a rare condition that is usually congenital in origin.1 It is often overlooked during clinical examination, routine echocardiography, and even at cardiac catheterization. Most of the reported cases were diagnosed at operation or autopsy because the diagnosis is not easy without appropriate investigation.1,2 It is almost invariably fatal in its bilateral and severe forms.3 Although characteristic echocardiographic and Doppler findings of pulmonary vein stenosis have been reported,4-8 few data were available regarding hemodynamic features during cardiac catheterization. This report describes the pulsed Doppler, color flow echocardiography, and hemodynamic findings of bilateral pulmonary vein stenosis that accompany ventricular septal defect and atrial septal defect.
CASE REPORT A 28-year-old female patient was admitted for shortness of breath. She had been dyspneic since birth and postponed surgery for personal reasons. The electrocardiogram revealed normal sinus rhythm, right axis deviation, and incomplete right bundle branch block. Physical examination revealed pansystolic murmur at the left lower sternal border with thrill. Chest radiograph revealed cardiomegaly and the prominent main pulmonary artery with cephalization and centralization of the pulmonary vasculature. Left pulmonary vein stenosis was suspected on the basis of the presence of a turbulent jet that originated from left pulmonary vein identified on the From the Cardiology Division and Department of Cardiothoracic Surgery, Yonsei Cardiovascular Center, Yonsei University College of Medicine. Reprint requests: Namsik Chung, MD, Cardiology Division, Yonsei Cardiovascular Center, Yonsei University, College of Medicine, CPO Box 8044, Seoul, Korea. Copyright © 1998 by the American Society of Echocardiography. 0894-7317/98 $5.00 1 0 27/4/88323
describes the pulsed Doppler, color flow echocardiography, and hemodynamic findings of bilateral pulmonary vein stenosis that accompany ventricular septal defect and atrial septal defect corrected by surgery. (J Am Soc Echocardiogr 1998;11:393-6.)
parasternal long-axis view (Figure 1, A). Right pulmonary vein stenosis was also suspected because of a similar turbulent jet observed on the apical four-chamber view (Figure 1, B). Pulsed wave Doppler interrogation of jets that originated from entry points of both pulmonary veins during transesophageal echocardiography demonstrated high peak velocity of 2.6 m/sec and 2.1 m/sec, respectively (Figure 2). Cardiac catheterization was performed with a 7F NIH catheter and an 8F Mullins sheath. At cardiac catheterization, the pulmonary artery pressure was 50/28 mm Hg. The left atrial mean pressure was 3 mm Hg, the mean left upper pulmonary vein pressure was 16 mm Hg, and the mean right upper pulmonary vein was 18 mm Hg (Figure 3). Oxymetry revealed significant oxygen step-up between mixed venous blood and the right atrium and between the right atrium and the right ventricle. A 7F NIH catheter and Mullins sheath were entered into the left atrium through the atrial septal defect, and selective cannulation of both pulmonary veins was performed with a 7F NIH catheter. Simultaneous pressure tracings made with a 7F NIH catheter and an 8F Mullins sheath revealed significant pressure gradients between the right upper pulmonary vein and left atrium and between the left upper pulmonary vein and left atrium. A selective angiography of right and left upper pulmonary veins with a 7F NIH catheter demonstrated a discrete narrowing at their entry sites into the left atrium (Figure 4). After cardiopulmonary bypass was established, the right atrium was opened and the pulmonary veins were inspected. There were fibrous membranelike structures obstructing the ostia of both upper pulmonary veins. After removal of the abnormal membranes, a Hegar dilator (No. 12) could easily be passed through both pulmonary veins. Patch repair of atrial septal defect and ventricular septal defect were performed. The gross and histologic findings were similar to those previously reported. It was a whitish, graycolored tissue with thick fibrosis and diffuse degeneration. 393
394 Ha et al.
Figure 1 Parasternal long-axis view (A) and modified apical four-chamber view (B) show turbulent color flow jets at the entrance of the right and left pulmonary veins into the left atrium. RV, Right ventricle; LV, left ventricle; AO, aorta; RA, right atrium.
DISCUSSION Pulmonary vein stenosis is a rare condition that is usually congenital in origin1 and is often overlooked during clinical examination, routine echocardiography, and even at cardiac catheterization. Obstruction of pulmonary venous flow may occur before or after surgery. Before surgery, total anomalous pulmonary venous return9 and cor triatriatum10 have been found to coexist with pulmonary vein stenosis. The clinical findings and radiographic features may mimic primary lung disease, and the diagnosis is frequently delayed or not made until autopsy is performed. Without preoperative identification and surgical correction of this anatomic abnormality, some patients are prone to pulmonary congestion after surgery. Highly sensitive and specific noninvasive anatomic diagnosis of all of these congenital anomalies has been made possible by two-dimensional echocardiography. Two-dimensional echocardiography alone, however, is not sufficient to detect this
Journal of the American Society of Echocardiography April 1998
Figure 2 Pulsed wave Doppler findings in right (A) and left (B) pulmonary vein stenosis by transesophageal echocardiography (TEE) before and after surgery revealed continuous high velocity flow, which decreased in peak velocity after surgery.
anomaly. Moreover, estimation of the severity of the functional abnormalities with this anomaly is not possible. Color Doppler imaging can easily demonstrate turbulent flow from the entry point of the pulmonary vein, thus suggesting obstruction. Samdarshi et al.6 reported transesophageal echocardiographic findings of pulmonary vein stenosis and concluded that transesophageal echocardiography is well suited for examination of the pulmonary veins and diagnosis of pulmonary venous obstruction. Frequency aliasing observed by transthoracic color Doppler imaging is also an important clue in the diagnosis of pulmonary vein stenosis in this report. Therefore, this particular transthoracic color Doppler echocardiographic finding may provide important diagnostic information on pulmonary vein stenosis, which is often overlooked before surgery. Doppler echocardiography can be used in the quantitative analysis of severity of functional abnormality. However, in some instances of increased
Journal of the American Society of Echocardiography Volume 11 Number 4
Ha et al. 395
Figure 3 Simultaneous pressure tracings of left atrium and right (A) and left (B) pulmonary veins revealed significant pressure gradients across the venoatrial junction. RUPV, Right upper pulmonary vein; LA, left atrium; LUPV, left upper pulmonary vein.
angle to flow, the actual pressure gradient produced by the obstruction may be underestimated. Definitive evaluation of the severity of such functional difficulties can be made by cardiac catheterization, but additional procedures or special skills, such as transseptal puncture or catheter manipulation are needed to steer the catheter across the intra-atrial baffle created by the Mustard procedure.11 In our patient, coexistence of atrial septal defect made it easy to measure the pressure gradient between the left atrial and both pulmonary veins and to visualize the sites of obstruction by selective angiography. In most instances, pulmonary artery wedge angiography is performed for the demonstration of the site of obstruction at cardiac catheterization.3
Figure 4 Selective angiograms of right upper pulmonary vein (RUPV) (A) and left upper pulmonary vein (LUPV) (B) demonstrate stenosis at the venoatrial junction. REFERENCES 1. Sade RM, Freed MD, Mathews EC, Castaneda AR. Stenosis of individual pulmonary veins: review of the literature and report of a surgical case. J Thorac Cardiovasc Surg 1974; 67:953-62. 2. Moller J, Noren G, David P, Amplatx K, Kanjuh V, Edwards J. Clinical pathologic conference. Am Heart J 1966; 73:530-7. 3. Bini RM, Cleveland DC, Ceballos R, Bargeron LM, Pacifico AD, Kirklin JW. Congenital pulmonary vein stenosis. Am J Cardiol 1984;54:369-75. 4. Smallhorn JF, Pauperio H, Benson L, Freedom RM, Rowe RD. Pulsed Doppler assessment of pulmonary vein obstruction. Am Heart J 1985;110:483-6.
396 Ha et al.
5. Dev V, Shrivastava S. Diagnosis of pulmonary venous obstruction by Doppler echocardiography. Int J Cardiol 1989;22:12933. 6. Samdarshi TE, Morrow WR, Helmcke FR, Nanda NC, Bargeron LM, Pacifico AD. Assessment of pulmonary vein stenosis by transesophageal echocardiography. Am Heart J 1991;122:1495-8. 7. Vick GW III, Murphy DJ II, Ludomirsky A, et al. Pulmonary venous and systemic ventricular inflow obstruction in patients with congenital heart disease: detection by combined twodimensional and Doppler echocardiography. J Am Coll Cardiol 1987;9:580-7.
Journal of the American Society of Echocardiography April 1998
8. Webber SA, de Souza E, Patterson MWH. Pulsed wave and color Doppler findings in congenital pulmonary vein stenosis. Pediatr Cardiol 1992;13:112-5. 9. Gathman GE, Nadas AS. Total anomalous pulmonary venous connection: clinical and physiologic observations of 75 pediatric patients. Circulation 1970;42:143-54. 10. Jegier W, Gibbons JE, Wiglesworth FW. Cor triatriatum: clinical, hemodynamic, and pathologic studies: surgical correction in early life. Pediatrics 1963;31:255-67. 11. Mullins CE. Transseptal left heart catheterization: experience with a new technique in 520 pediatric and adult patients. Pediatr Cardiol 1983;4:239-45.
Pulmonary vein stenosis is a rare condition that usually is congenital in origin; it is almost invariably fatal in its bilateral and severe forms. It is often overlooked, however, during clinical examination, routine echocardiography, and even at cardiac catheterization. This report
P
ulmonary vein stenosis is a rare condition that is usually congenital in origin.1 It is often overlooked during clinical examination, routine echocardiography, and even at cardiac catheterization. Most of the reported cases were diagnosed at operation or autopsy because the diagnosis is not easy without appropriate investigation.1,2 It is almost invariably fatal in its bilateral and severe forms.3 Although characteristic echocardiographic and Doppler findings of pulmonary vein stenosis have been reported,4-8 few data were available regarding hemodynamic features during cardiac catheterization. This report describes the pulsed Doppler, color flow echocardiography, and hemodynamic findings of bilateral pulmonary vein stenosis that accompany ventricular septal defect and atrial septal defect.
CASE REPORT A 28-year-old female patient was admitted for shortness of breath. She had been dyspneic since birth and postponed surgery for personal reasons. The electrocardiogram revealed normal sinus rhythm, right axis deviation, and incomplete right bundle branch block. Physical examination revealed pansystolic murmur at the left lower sternal border with thrill. Chest radiograph revealed cardiomegaly and the prominent main pulmonary artery with cephalization and centralization of the pulmonary vasculature. Left pulmonary vein stenosis was suspected on the basis of the presence of a turbulent jet that originated from left pulmonary vein identified on the From the Cardiology Division and Department of Cardiothoracic Surgery, Yonsei Cardiovascular Center, Yonsei University College of Medicine. Reprint requests: Namsik Chung, MD, Cardiology Division, Yonsei Cardiovascular Center, Yonsei University, College of Medicine, CPO Box 8044, Seoul, Korea. Copyright © 1998 by the American Society of Echocardiography. 0894-7317/98 $5.00 1 0 27/4/88323
describes the pulsed Doppler, color flow echocardiography, and hemodynamic findings of bilateral pulmonary vein stenosis that accompany ventricular septal defect and atrial septal defect corrected by surgery. (J Am Soc Echocardiogr 1998;11:393-6.)
parasternal long-axis view (Figure 1, A). Right pulmonary vein stenosis was also suspected because of a similar turbulent jet observed on the apical four-chamber view (Figure 1, B). Pulsed wave Doppler interrogation of jets that originated from entry points of both pulmonary veins during transesophageal echocardiography demonstrated high peak velocity of 2.6 m/sec and 2.1 m/sec, respectively (Figure 2). Cardiac catheterization was performed with a 7F NIH catheter and an 8F Mullins sheath. At cardiac catheterization, the pulmonary artery pressure was 50/28 mm Hg. The left atrial mean pressure was 3 mm Hg, the mean left upper pulmonary vein pressure was 16 mm Hg, and the mean right upper pulmonary vein was 18 mm Hg (Figure 3). Oxymetry revealed significant oxygen step-up between mixed venous blood and the right atrium and between the right atrium and the right ventricle. A 7F NIH catheter and Mullins sheath were entered into the left atrium through the atrial septal defect, and selective cannulation of both pulmonary veins was performed with a 7F NIH catheter. Simultaneous pressure tracings made with a 7F NIH catheter and an 8F Mullins sheath revealed significant pressure gradients between the right upper pulmonary vein and left atrium and between the left upper pulmonary vein and left atrium. A selective angiography of right and left upper pulmonary veins with a 7F NIH catheter demonstrated a discrete narrowing at their entry sites into the left atrium (Figure 4). After cardiopulmonary bypass was established, the right atrium was opened and the pulmonary veins were inspected. There were fibrous membranelike structures obstructing the ostia of both upper pulmonary veins. After removal of the abnormal membranes, a Hegar dilator (No. 12) could easily be passed through both pulmonary veins. Patch repair of atrial septal defect and ventricular septal defect were performed. The gross and histologic findings were similar to those previously reported. It was a whitish, graycolored tissue with thick fibrosis and diffuse degeneration. 393
394 Ha et al.
Figure 1 Parasternal long-axis view (A) and modified apical four-chamber view (B) show turbulent color flow jets at the entrance of the right and left pulmonary veins into the left atrium. RV, Right ventricle; LV, left ventricle; AO, aorta; RA, right atrium.
DISCUSSION Pulmonary vein stenosis is a rare condition that is usually congenital in origin1 and is often overlooked during clinical examination, routine echocardiography, and even at cardiac catheterization. Obstruction of pulmonary venous flow may occur before or after surgery. Before surgery, total anomalous pulmonary venous return9 and cor triatriatum10 have been found to coexist with pulmonary vein stenosis. The clinical findings and radiographic features may mimic primary lung disease, and the diagnosis is frequently delayed or not made until autopsy is performed. Without preoperative identification and surgical correction of this anatomic abnormality, some patients are prone to pulmonary congestion after surgery. Highly sensitive and specific noninvasive anatomic diagnosis of all of these congenital anomalies has been made possible by two-dimensional echocardiography. Two-dimensional echocardiography alone, however, is not sufficient to detect this
Journal of the American Society of Echocardiography April 1998
Figure 2 Pulsed wave Doppler findings in right (A) and left (B) pulmonary vein stenosis by transesophageal echocardiography (TEE) before and after surgery revealed continuous high velocity flow, which decreased in peak velocity after surgery.
anomaly. Moreover, estimation of the severity of the functional abnormalities with this anomaly is not possible. Color Doppler imaging can easily demonstrate turbulent flow from the entry point of the pulmonary vein, thus suggesting obstruction. Samdarshi et al.6 reported transesophageal echocardiographic findings of pulmonary vein stenosis and concluded that transesophageal echocardiography is well suited for examination of the pulmonary veins and diagnosis of pulmonary venous obstruction. Frequency aliasing observed by transthoracic color Doppler imaging is also an important clue in the diagnosis of pulmonary vein stenosis in this report. Therefore, this particular transthoracic color Doppler echocardiographic finding may provide important diagnostic information on pulmonary vein stenosis, which is often overlooked before surgery. Doppler echocardiography can be used in the quantitative analysis of severity of functional abnormality. However, in some instances of increased
Journal of the American Society of Echocardiography Volume 11 Number 4
Ha et al. 395
Figure 3 Simultaneous pressure tracings of left atrium and right (A) and left (B) pulmonary veins revealed significant pressure gradients across the venoatrial junction. RUPV, Right upper pulmonary vein; LA, left atrium; LUPV, left upper pulmonary vein.
angle to flow, the actual pressure gradient produced by the obstruction may be underestimated. Definitive evaluation of the severity of such functional difficulties can be made by cardiac catheterization, but additional procedures or special skills, such as transseptal puncture or catheter manipulation are needed to steer the catheter across the intra-atrial baffle created by the Mustard procedure.11 In our patient, coexistence of atrial septal defect made it easy to measure the pressure gradient between the left atrial and both pulmonary veins and to visualize the sites of obstruction by selective angiography. In most instances, pulmonary artery wedge angiography is performed for the demonstration of the site of obstruction at cardiac catheterization.3
Figure 4 Selective angiograms of right upper pulmonary vein (RUPV) (A) and left upper pulmonary vein (LUPV) (B) demonstrate stenosis at the venoatrial junction. REFERENCES 1. Sade RM, Freed MD, Mathews EC, Castaneda AR. Stenosis of individual pulmonary veins: review of the literature and report of a surgical case. J Thorac Cardiovasc Surg 1974; 67:953-62. 2. Moller J, Noren G, David P, Amplatx K, Kanjuh V, Edwards J. Clinical pathologic conference. Am Heart J 1966; 73:530-7. 3. Bini RM, Cleveland DC, Ceballos R, Bargeron LM, Pacifico AD, Kirklin JW. Congenital pulmonary vein stenosis. Am J Cardiol 1984;54:369-75. 4. Smallhorn JF, Pauperio H, Benson L, Freedom RM, Rowe RD. Pulsed Doppler assessment of pulmonary vein obstruction. Am Heart J 1985;110:483-6.
396 Ha et al.
5. Dev V, Shrivastava S. Diagnosis of pulmonary venous obstruction by Doppler echocardiography. Int J Cardiol 1989;22:12933. 6. Samdarshi TE, Morrow WR, Helmcke FR, Nanda NC, Bargeron LM, Pacifico AD. Assessment of pulmonary vein stenosis by transesophageal echocardiography. Am Heart J 1991;122:1495-8. 7. Vick GW III, Murphy DJ II, Ludomirsky A, et al. Pulmonary venous and systemic ventricular inflow obstruction in patients with congenital heart disease: detection by combined twodimensional and Doppler echocardiography. J Am Coll Cardiol 1987;9:580-7.
Journal of the American Society of Echocardiography April 1998
8. Webber SA, de Souza E, Patterson MWH. Pulsed wave and color Doppler findings in congenital pulmonary vein stenosis. Pediatr Cardiol 1992;13:112-5. 9. Gathman GE, Nadas AS. Total anomalous pulmonary venous connection: clinical and physiologic observations of 75 pediatric patients. Circulation 1970;42:143-54. 10. Jegier W, Gibbons JE, Wiglesworth FW. Cor triatriatum: clinical, hemodynamic, and pathologic studies: surgical correction in early life. Pediatrics 1963;31:255-67. 11. Mullins CE. Transseptal left heart catheterization: experience with a new technique in 520 pediatric and adult patients. Pediatr Cardiol 1983;4:239-45.