Diagnostic Value of Transesophageal Echocardiography in Platypnea
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Diagnostic Value of Transesophageal Echocardiography in Platypnea Marie-Christine Herregods, MD, Car! Timmermans, MD, Eric Frans, MD, Marc Decramer, MD, Wim Daenen, MD, and Hilaire De Geest, MD, Leuven, Belgium Platypnea is a rare syndrome of orthostatic dyspnea frequently caused by an interatrial right-to-left shunt. The diagnosis is difficult. Assessment of arterial blood gases reveals orthostatic desaturation. In the past, definite diagnosis necessitated catheterization in the supine and upright position. Now transesophageal echocardiography on a tilt table combined with a peripheral venous contrast study provides correct diagnosis in a safe and easy way. (JAM Sac EcHOCARDIOGR 1993;6:624-7.) Platypnea is dyspnea that exacerbates in the upright position and relieves in the supine position. This rare syndrome has been observed after pulmonary artery embolism, in pulmonary arteriovenous fistulae, in se- vere chronic lung disease, and after thoracotomy. 1 • 2 All reported cases of postthoracotomy platypnea were caused by righno-left shunting across the in- teratrial septum (patent foramen ovale, secundum- type atrial septal defect) without evidence of pul- monary hypertension. 1 The pathophysiologic cause is still unknown. The initial examination in the diagnosis of platyp- nea is measurement of arterial blood gases. 3 Ortho- static desaturation is pathognomonic and obligates further investigation. The case described below il- lustrates the usefulness of transesophageal echocar- diography in obtaining a definite diagnosis. CASE REPORT A 66-year-old man was readmitted to the hospital complaining of extreme dyspnea 2 months after right pneumonectomy because of a spinocellular ep- ithelioma. Physical examination was normal. The left lung was clear by auscultation. Heart sounds were normal and no murmur was heard. Blood pressure varied within normal limits. The electrocardiogram showed sinus tachycardia. On chest X-ray film, the From the Department of Cardiology, Pneumology, and Cardiac Surgery, University Hospital Gasthuisberg, Leuven, Belgium. Reprint requests: M.C. Herregods, Department of Cardiology, UZ Gasthuisberg, Herestraat 49, B-3000 Leuven, Belgium. Copyright© 1993 by the American Society ofEchocardiography. 0894-7317/93 $1.00 + .10 27/l/47825 624 left lung was normal with hydrothorax on the right side and rightward shift of the mediastinum. Pul- monary function studies were as expected for a pa- tient after pneumonectomy. Arterial blood gas levels were as follows: in the supine position Po 2 was 57 mm Hg, Pco 2 was 29 mm Hg (3.5 L oxygen per minute), and Po 2 was 337 mm Hg (lOO% oxygen); in the sitting position Po 2 was 33 mm Hg, Pco 2 was 25 mm Hg (3.5 L oxygen per minute), and Po 2 was 72 mm Hg (lOO% oxygen). Because transthoracic echocardiography was tech- nically difficult because of displacement of the heart, a transesophageal echocardiogram was performed. By use of a tilt table, the patient could be examined in the supine and upright positions. The right atrium was small. There were no signs of extracardiac compression. An atrial septal aneurysm bulging into the left atrium was prominent (Figure 1). A periph- eral venous contrast study with saline solution in- jected into the arm revealed a right-to-left shunt at the level of the atrial septal aneurysm (Figure 2). This shunt was clearly increasing in the upright position (Figure 3). The diameters of the right atrium and ventricle and their anatomic relationship to the left side of the heart did not change. Subsequently a cardiac catheterization in the usual recumbent position was performed confirming the atrial septal aneurysm and right-to-left shunt. Intra- cardiac pressures were completely normal. At surgery an atrial septal aneurysm (with two small defects of nearly 2 mm) was found and resected. A repair by primary closure was done. After surgery there was complete symptomatic re- lief. A control transesophageal echocardiogram dem- onstrated normal dimensions of right atrium and ventricle (Figure 4). During saline solution contrast study, echoes appeared in the right atrium and ven-
Transcript of Diagnostic Value of Transesophageal Echocardiography in Platypnea
Diagnostic Value of Transesophageal Echocardiography in Platypnea
Marie-Christine Herregods, MD, Car! Timmermans, MD, Eric Frans, MD, Marc Decramer, MD, Wim Daenen, MD, and Hilaire De Geest, MD, Leuven, Belgium
Platypnea is a rare syndrome of orthostatic dyspnea frequently caused by an interatrial right-to-left shunt. The diagnosis is difficult. Assessment of arterial blood gases reveals orthostatic desaturation. In the past, definite diagnosis necessitated catheterization in the supine and upright position. Now transesophageal echocardiography on a tilt table combined with a peripheral venous contrast study provides correct diagnosis in a safe and easy way. (JAM Sac EcHOCARDIOGR 1993;6:624-7.)
Platypnea is dyspnea that exacerbates in the upright position and relieves in the supine position. This rare syndrome has been observed after pulmonary artery embolism, in pulmonary arteriovenous fistulae, in severe chronic lung disease, and after thoracotomy. 1
•2
All reported cases of postthoracotomy platypnea were caused by righno-left shunting across the interatrial septum (patent foramen ovale, secundumtype atrial septal defect) without evidence of pulmonary hypertension. 1 The pathophysiologic cause is still unknown.
The initial examination in the diagnosis of platypnea is measurement of arterial blood gases. 3 Orthostatic desaturation is pathognomonic and obligates further investigation. The case described below illustrates the usefulness of transesophageal echocardiography in obtaining a definite diagnosis.
CASE REPORT
A 66-year-old man was readmitted to the hospital complaining of extreme dyspnea 2 months after right pneumonectomy because of a spinocellular epithelioma. Physical examination was normal. The left lung was clear by auscultation. Heart sounds were normal and no murmur was heard. Blood pressure varied within normal limits. The electrocardiogram showed sinus tachycardia. On chest X-ray film, the
From the Department of Cardiology, Pneumology, and Cardiac Surgery, University Hospital Gasthuisberg, Leuven, Belgium. Reprint requests: M.C. Herregods, Department of Cardiology, UZ Gasthuisberg, Herestraat 49, B-3000 Leuven, Belgium. Copyright© 1993 by the American Society ofEchocardiography. 0894-7317/93 $1.00 + .10 27/l/47825
624
left lung was normal with hydrothorax on the right side and rightward shift of the mediastinum. Pulmonary function studies were as expected for a patient after pneumonectomy. Arterial blood gas levels were as follows: in the supine position Po2 was 57 mm Hg, Pco2 was 29 mm Hg (3.5 L oxygen per minute), and Po2 was 337 mm Hg (lOO% oxygen); in the sitting position Po2 was 33 mm Hg, Pco2 was 25 mm Hg (3.5 L oxygen per minute), and Po2 was 72 mm Hg (lOO% oxygen).
Because transthoracic echocardiography was technically difficult because of displacement of the heart, a transesophageal echocardiogram was performed. By use of a tilt table, the patient could be examined in the supine and upright positions. The right atrium was small. There were no signs of extracardiac compression. An atrial septal aneurysm bulging into the left atrium was prominent (Figure 1). A peripheral venous contrast study with saline solution injected into the arm revealed a right-to-left shunt at the level of the atrial septal aneurysm (Figure 2). This shunt was clearly increasing in the upright position (Figure 3). The diameters of the right atrium and ventricle and their anatomic relationship to the left side of the heart did not change.
Subsequently a cardiac catheterization in the usual recumbent position was performed confirming the atrial septal aneurysm and right-to-left shunt. Intracardiac pressures were completely normal.
At surgery an atrial septal aneurysm (with two small defects of nearly 2 mm) was found and resected. A repair by primary closure was done.
After surgery there was complete symptomatic relief. A control transesophageal echocardiogram demonstrated normal dimensions of right atrium and ventricle (Figure 4). During saline solution contrast study, echoes appeared in the right atrium and ven-
Journal of the American Society of Echocardiography Volume 6 Number 6 Herregods et al. 625
Figure 1 Visualization of an atrial septal aneurysm type II and small right atrium. ASA., Atrial septal aneurysm; AO, aorta; LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle.
Figure 2 Peripheral venous contrast study: demonstration of right-to-left shunting at the level of the atrial septal aneurysm. ASA., Atrial septal aneurysm; RA, right atrium; LA, left atrium; AO, aorta; LV, left ventricle.
tricle only, in the supine and upright position (Figure 5).
DISCUSSION
To our knowledge, this is the first description of platypnea caused by right-to-left shunting through an atrial septal aneurysm and diagnosed by transesophageal echocardiography.
Transesophageal echocardiography is an ideal method to evaluate the interatrial septum. Atrial septal aneurysm is easily recognized. 4 Moreover a transesophageal echocardiogram can be performed on a tilt table without technical difficulties. In our case a peripheral venous contrast study with saline solution revealed in the supine position a right-to-left shunt at the level of the atrial septal aneurysm; the shunt was obviously increased in the upright position.
The pathophysiologic cause of platypnea is still
626 Herregods et al. Journal of the American Society of Echocardiography
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Figure 3 Increase of right-to-left atrial shunting in the upright position. ASAJ Atrial septal aneurysm; RAJ right atrium; IAJ left atrium; AO) aorta; LV) left ventricle.
Figure 4 Intact atrial septum after surgery and normal dimensions of right atrium. LA) Left atrium; AS) atrial septum; AOJ aorta; LV) left ventricle; RV, right ventricle.
unknown. Since 1949 a few cases of platypnea after right pneumonectomy are described. 5 Pulmonary hypertension or a pressure gradient at the atrial level has never been reported. Some authors assumed a reduction in intravascular volume and decreased right ventricular compliance.6 Others have mentioned a mechanical factor with distortion of the fossa ovalis
and abnormal anatornic relationship of one of the venae cavae to the septal defect. 7-
9
Our echocardiographic findings suggest a mechanical influence. The right atrium was smaller than expected. However, extracardiac compression could not be observed. Although a manifest increase of the right-to-left shunt occurred in the upright position,
J oumal of the American Society of Echocardiography Volume 6 Number 6 Herregods et al. 627
Figure 5 Postoperative venous contrast study: no shunting at the atrial level. LA) Left atrium; AS) atrial septum; AO) aorta; RAJ right atrium; RV) right ventricle.
the dimensions and relative position of the cardiac chambers did not change. After surgical removal of the interatrial septal aneurysm, the position and dimensions of the cardiac chambers were normal.
We believe that transesophageal echocardiography is the ideal method to correctly diagnose platypnea. Moreover it provides the opportunity to investigate the mechanism of platypnea by comparing the examinations before pneumonectomy with those after pneumonectomy.
REFERENCES
l. Seward JB, Hayes DL, Smith HC, et al. Platypnea-orthodeoxia: clinical profile, diagnostic workup, management, and report of seven cases. Mayo Clin Proc 1984;59:221-31.
2. Springer RM, Gheorghiade M, Chakko CS, et al. Platypnea and interatrial right-to-left shunting after lobectomy. Am J Cardiol1983;51 : 1802-3.
3. Schnabel TG, Rarto 0, Kirby CK,)ohnson J, Comroe JH. Postural cyanosis and angina pectoris following pneumonectomy: relief by closure of an interatrial septal defect. J Thorac Cardiovasc Surg 1956;32(2) :246-50.
4. Schneider B, Hoftnann T, Meinertz T, Hanrath P. Diagnostic value of transesophageal echocardiography in atrial septal aneurysm. lnt J Card Imaging 1992;8:143-52.
5. Selzer A, Lewis AE. The occurrence of chronic cyanosis in cases of atrial septal defect. Am J Med Sci 1949;218:516-24.
6. Labresh KA, Pietro DA, Coates EO, Khuri SF, Folland ED, Parisi AF. Platypnea syndrome after left pneumonectomy. Chest 1981;79:605-7.
7. Dlabal PW, Sturts BS, Jenkins DW, Harkleroad LE, Stanford WT. Cyanosis following right pneumonectomy. Importance of patent foramen ovale. Chest 1982;81:370-2.
8. Franco DP, Kinasewitz GT, Markham RV, Tucker WY, George RB. Postural hypoxemia in the postpneumonectomy patient. Am Rev Respir Dis 1984;129:1021-2.
9. Adolph EA, Lacy WO, Hermoni YI, Wexler LF, Javaheri S. Reversible orthodeoxia and platypnea due to right-to-left intracardiac shunting related to pericardial effusion. Ann Intern Med 1992;16:138-9.
