Pediatr Cardiol 26:700702, 2005 DOI: 10.

1007/s00246-005-0893-8

Unilateral Pulmonary Edema: Unusual Presentation of Acute Rheumatic Fever

A. EI-Menyar,1 A. AI-Hroob,2 M.T. Numan,2 S.M. Gendi,2 I.M. Fawzy3
1 2 3

Departments of Cardiology and Cardiovascular Surgery, Hamad General Hospital, P.O. Box 3050, Doha, Qatar Department of Pediatric Cardiology, Hamad General Hospital, P.O. Box 3050, Doha, Qatar Department of Internal Medicine, Hamad General Hospital, P.O. Box 3050, Doha, Qatar

Abstract. Cardiogenic unilateral pulmonary edema (UPE) is a rare clinical condition and not readily recognized early and managed accordingly. Acute rheumatic fever, which is a common disease in developing countries, does not commonly present with UPE. We report a 13-year-old girl presenting with UPE following acute rheumatic fever mimicking pneumonia. We conclude that UPE should be considered in the dierential diagnosis for the patient with clinical criteria of rheumatic fever who presents with unilateral lung opacication. With early recognition and antifailure treatment, it is possible to reduce morbidity and mortality in such patients. Key words: Unilateral pulmonary edema Rheumatic fever

Chest X-ray showed a clear left lung, whereas the right lung showed patchy alveolar inltrates. A provisional diagnosis of right-sided pneumonia was entertained, and chest tomography scan revealed right-sided pneumnitis versus interstitial edema (Fig. 1). There was no response to empirical antibiotic treatment. While in the hospital, respiratory distress worsened. Lung congestion, which was worse on the right lung, became bilateral. Further clinical and echocardiographic evaluation proved severe congestive heart failure secondary to mitral regurgitation with ail mitral leaet resulting from ruptured chorde tendenae in the course of the acute rheumatic carditis (Fig. 2). No vegetations were detected and her blood cultures were repeatedly negative. She responded dramatically to furesmide and milirenone infusion in addition to digitalis, captopril, aspirin, and steroids.

Discussion Unilateral pulmonary edema (UPE) is an uncommon presentation of acute rheumatic fever. It has been reported as a clinical manifestation of post-streptococcal glomerulonephritis without valvular aection [11]. The underlying mechanism of heart failure due to poststreptococcal glomerulonephritis is dierent than that of poststreptococcal carditis [5, 11]. Pulmonary edema associated with mitral valve regurgitation is usually bilateral and cardiac in origin. However, occasionally it is isolated or predominantly right upper lobe pulmonary edema [1, 3, 10, 19]. The initial diagnosis of pneumonia is often made incorrectly since the possibility of cardiac disease is not considered. The direction of the regurgitant jet caused by ail mitral valve leaets varies depending on the leaet involved, and the jet of regurgitant ow in a patient with a ail posterior valve leaet is directed toward the right pulmonary vein, which is the case in the patient presented here [1517]. Schnyder and coworkers [17] reported a 9% prevalence of predominantly right upper lobe pulmonary edema in cases of severe mitral valve regurgitation. This emphasizes the need for the dierential diagnosis of isolated right upper lung edema to include mitral valve disease. Further evidence of a pressure dierential between the

Case Report
A 13-year-old girl presented to the emergency department with a history of progressive cough and shortness of breath 2 days in duration. She had bilateral ankle arthritis and a sore throat 1 month earlier and a history of recurrent tonsillitis, for which elective tonsillectomy was performed 3 years prior to this illness. On physical examination, she appeared ill, with a temperature of 38C, heart rate 100 beats/min, respiratory rate 38/min, and blood pressure 100/60 mmHg. Her jugular venous pulsation was normal; chest examination revealed right-sided diuse ne inspiratory crackles. Cardiac examination showed pansystolic murmur on the apex radiated all over the precordium as well as diastolic murmur of the aortic area. These ndings were suggestive of mitral and aortic incompetence. Her right ankle was swollen, red, and tender. Blood count showed the following: Hb, 10.9 g/dl; white blood cell count, 13.000 (71% granulocytes); mean corpuscular volume (MCV), 78, hematocrit, 32.8; platelet, 408,000; erythrocyte sedimentation rate (ESR), 111 mm/h; elevated Anti-Streptohygin-oTiter (ASO) titer, 250 IU. Arterial blood gas analysis at room air revealed a pH of 7.4, PCO2 30 mmHg, and Pa2 90 mmHg. Her serum electrolyte, creatinine, and liver enzyme levels were normal. Correspondence to: A. EI-Menyar, email: aymanco65@yahoo.com

EI-Menyar et al: Unilateral Pulmonary Edema

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Fig. 1. (A) Chest X-ray shows whitish patch shadow in the right lung on arrival. (B) Tomography scan of chest on admission. (C) Chest X-ray after diuretic and amirinone infusion. RT, right.

Fig. 2. (A and B) Transthoracic and transesophageal echocardiography show systolic incoapted mitral valve due to ail posterior leaet. (C and D) Color transesophageal image shows the severity of mitral regurgitation and its eccentric pattern hugging the atrial wall and right pulmonary vein. (E and F) Pulsed Doppler tracing for the right pulmonary vein (RPV) and left pulmonary vein (LPV).

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right and left pulmonary veins conrms the nding. Therefore, right upper lobe edema is most likely the result of an asymmetric increase in the hydrostatic pressure of the right upper lung caused by asymmetric blood ow from the left atrium. Common causes of unilateral pulmonary inltrates in children are pneumonia, atelectasis, and pulmonary hemorrhage. UPE associated with ipsilateral pulmonary lesions is rare but has been reported, especially following rapid removal of pleural air or eusions [2, 4, 6-9, 12]. Rapid reexpansion of the collapsed lung causes a prompt increase in blood ow and pulmonary capillary pressure and precipitates pulmonary edema. Protracted lateral decubitus posture, hyperperfusion but hypoventilation of the dependent lobe during general anesthesia, concomitant uid overloading, and decompensated ventricular function predispose to UPE [13, 18]. Valvular heart disease with left ventricular failure, SwyerJames syndrome, infusion through a misplaced intravenous catheter into a branch of the pulmonary artery, obstruction of a bronchus by a foreign body, or even laryngospasm can cause UPE [6, 16]. Neither physical nor radiographic examination of the chest allow easy discrimination of infectious versus uid-related inltrates. Radiographic abnormalities were discovered in 5685% of patients with glomerulonephritis, and cardiomegaly is common in approximately half of the patients [l1, 14]. The cause of UPE was not apparent in our patient because she had no clinical history consistent with previous unilateral lung disease, no history of prolonged lateral decubitus position, no hydrothorax or pneumothorax, and no invasive catheter insertion. Our patient developed UPE on the right lung with progressive bilateral involvement within 48 hours. Complete and prompt clearance of edema and resolution of cardiomegaly after anti-failure therapy suggest that unilaterality is an early manifestation of bilateral pulmonary edema. We conclude that UPE may be part of the presentation of acute rheumatic fever post-streptococcus pharyngitis secondary to severe acute mitral regurgitation resulting from ruptured chorda and ail mitral leaet. Conclusion UPE should be considered in the dierential diagnosis of patients with clinical criteria of rheumatic fever who present with unilateral lung opacication. This will greatly impact therapeutic measures to reduce morbidity and mortality in these patients.

References
1. Alarcon JJ, Guembe P, de Miguel E, Gordillo I, Abellas A (1995) Localized right upper lobe edema. Chest 107: 274276 2. Audenaert SM (1993) Unilateral pulmonary edema in children. Clin Pediatr 32:363365 3. Bahl OP, Oliver GC, Rocko SD, Parker BM (1971) Localized unilateral pulmonary edema: an unusual presentation of left heart failure. Chest 60:277280 4. Bourke AM (1997) Unilateral pulmonary oedema following postextubation laryngospasm. Anaesthesia 52:928 5. Brimacombe J, Laxton C (1995) Myocardial dysfunction in a 23-month child with acute poststreptococcal glomerulonephritis. Anaesth Intensive Care 23:222224 6. Caleno L, Kruglik GD, Woodru A (1978) Unilateral pulmonary edema. Radiology l26:1924 7. Cascade PN, Alexander GD, Mackie DS (1993) Negative pressure pulmonary oedema after endotracheal intubation. Radiology 186:671675 8. Chang KW, Wong KS, Wang JW, et al. (1995) BiPAP mask ventilation for expansion pulmonary oedema. Clin Intensive Care 6:293295 9. Gabbott DA, Gregory M (1997) Unilateral pulmonary oedema following laryngospasm. Anaesth Intenive Care 25:550 552 10. Gurney JW, Goodman LR (1989) Pulmonary edema localized in the right upper lobe accompanying mitral regurgitation. Radiology 171:397399 11. Kin S W, Ghi J L, Ching H L, Reyin L (2003) Unilateral pulmonary edema: an uncommon presentation of poststreptococcal glomerulonephritis. Pediatr Emerg Care 19:337339 12. Kramer MR, Melzer E, Sprung CL (1989) Unilateral pulmonary edema after intubation of the right main stem bronchus. Crit Care Med 17(5): 472474 13. Liers G, Umbrain V, Lamote J, et al. (2000) unilateral lung edema during anesthesia for reconstructive surgery of the trachea after caustic agent ingestion. J Cardiothorac Vasc Surg 14:8286 14. Macpherson SI, Banerjee AK (1974) Acute glomerulonephritis: a chest lm diagnosis? J Assoc Can Radiologists 25:5864 15. Miyatake K, Nimura Y, Sakakibara H, et al. (1982) Localisation and direction of mitral regurgitant ow in mitral orice studied with combined use of ultrasonic pulsed Doppler technique and two dimensional echocardiography. Br Heart J 48:449581 16. Roach JM, Stajduhar KC, Torrington KG (1993) Rjght upper lobe pulmonary edema caused by acute mitral regurgitation. Diagnosis by transesophageal echocardiography. Chest 103:12861288 17. Schnyder PA, Sarraj AM, Duvoisin BE, Kapenberger L, Landry MJ (1993) Pulmonary edema associated with mitral regurgitation: prevalence of predominant involvement of the right upper lobe. Am J Roentgenol 161:3336 18. Tsai YS, Wang SJ, Shih HC, et al. (1997) Unilateral pulmonary edema during general anesthesia report of two cases. Acta Anaesthesiol Sin 35:175180 19. Woolley K, Stark P (1999) Pulmonary parenchymal manifestations of mitral valve disease. Radiographics 19: 965972