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Introduction

Unilateral cardiogenic pulmonary ede-ma is a rare clinical and radiological pre-sentation of acute dyspnea and it is oftenconfused with other most common causesof unilateral alveolar and interstitial infil-trates such as infection, lung cancer, lunginfarction, atelectasis, aspiration andbronchial obstruction. We describe a caseof right pulmonary edema in a youngwoman with hypertrophic cardiomyopathyin whom the unilateral distribution of thelung involvement caused a delay in the cor-rect diagnosis and in the appropriate treat-ment. The rapid determination of two bio-logical markers, such as brain natriureticpeptide (BNP) and carbohydrate antigen125 (CA-125), frequently resulted elevatedin heart failure1,2, was helpful for the finaldiagnosis of cardiogenic pulmonary ede-ma.

Case report

A 27-year-old woman affected by non-obstructive hypertrophic cardiomyopathywas admitted to the emergency room of ourhospital for severe dyspnea associated withatrial tachycardia lasting for several hours.

Hypertrophic cardiomyopathy was di-agnosed when she was 14 years old and herprevious medical history was characterized

by recurrent syncopal episodes and conse-quent automatic cardioverter-defibrillatorimplantation. In the last years she reportedrecurrent episodes of atrial tachycardia,and she has been treated with metoprolol(200 mg/day), propafenone (600 mg/day)and amiodarone (200 mg/day).

On admission the patient complained ofpalpitation and dyspnea at rest. Heart ratewas 130 b/min, blood pressure 105/70mmHg, without fever.

Examination of the chest was normaland heart evaluation revealed tachycardiaand mild apical systolic murmur. Electro-cardiography showed atrial tachycardiawith heart rate of 140 b/min, without anysignificant signs of left ventricular hyper-trophy. Normal sinus rhythm occurred 12hours after admission, after successfuloverdrive pacing.

Hemoglobin, D-dimer, renal and hepat-ic function, serum electrolytes, creatine ki-nase-MB and troponin levels were withinnormal ranges. Chest roentgenographydocumented cardiomegaly and the pres-ence of the implanted automatic cardiovert-er-defibrillator, without signs of pulmonarycongestion.

Echocardiography, performed on hospi-tal admission, showed left ventricular asym-metric hypertrophy mainly affecting the in-terventricular septum (end-diastolic thick-ness 22 mm, left ventricular end-diastolic di-ameter 49 mm, left ventricular end-systolic

Key words:Brain natriureticpeptide; Hypertrophiccardiomyopathy;Pulmonary edema.

© 2005 CEPI Srl

Received February 2,2005; revision receivedApril 1, 2005; acceptedApril 4, 2005.

Address:

Dr. Antonio D’Aloia

Via Trieste, 7225121 BresciaE-mail:faggiano@numerica.it

A difficult diagnosis: right unilateralcardiogenic pulmonary edema. Usefulnessof biochemical markers of heart failurefor the correct diagnosisAntonio D’Aloia, Pompilio Faggiano*, Loretta Brentana, Alessandra Boldini,Roberto Procopio, Marco Racheli, Livio Dei Cas

Division of Cardiology, *Unit of Polycardiography, University of Brescia, Brescia, Italy

We describe a case of unilateral pulmonary edema occurring in a young woman affected by hy-pertrophic cardiomyopathy complicated by acute worsening of mitral regurgitation. The relevantrole of biochemical markers of heart failure, such as brain natriuretic peptide and carbohydrate anti-gen 125, in clarifying the final diagnosis of cardiogenic pulmonary edema and modifying treatmentaccordingly is emphasized.

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diameter 28 mm), anterior and lateral free walls; the leftatrium was markedly enlarged (50 � 90 mm, area 40cm2). Left ventricular systolic function was normal(ejection fraction > 60%). There was a mild mitral valveprolapse and moderate mitral regurgitation; there was nosign of intraventricular dynamic obstruction at basalconditions. Left ventricular filling pressure was typicalof abnormal relaxation (E/A wave ratio < 1, decelerationtime > 220 ms); indirect estimate of pulmonary systolicartery pressure was within the normal range.

Thirty-six hours later the patient suffered fromworsening dyspnea, fever (38°C), anxiety, hypoten-sion, and severe respiratory failure.

Chest roentgenography showed diffuse interstitialinfiltrates in the right lung (Fig. 1) in presence of leuko-cytosis (white blood cell count 12 000/mm3) and C-re-active protein level elevation. A chest computed to-mography confirmed the presence of interstitial infil-tration of the right lung and initial involvement of theleft upper lobe (Fig. 2).

Based on clinical (particularly fever), biochemical(abnormal white blood cell count) and imaging data, adiagnosis of pneumonia was presumed and, according-ly, broad-spectrum antibiotic therapy was started. De-tailed microbiological and serological investigationwas performed and resulted negative.

Three days later, due to persistence of a continuousstate of severe respiratory failure and monolateral rightinfiltrates at the second chest X-ray and computed to-mographic scan, with no evidence of infections or sep-sis and with antibiotic therapy inefficacy, serum levelsof BNP and CA-125 were determined: BNP was 1352pg/ml (normal values < 100 pg/ml) and CA-125 was 95U/ml (normal values < 30 U/ml) suggesting the pres-ence of heart failure.

Echocardiographic examination was then repeatedand showed, compared to that performed on admission,a more evident systolic anterior motion of the mitral an-terior leaflet with a 30 mmHg late-peaking intraven-tricular gradient. Furthermore, there was a worseningof mitral regurgitation, which was severe, with a colorDoppler flow jet directed toward the right pulmonaryveins (Fig. 3); left ventricular filling pattern was re-strictive (early filling to atrial systole ratio 2.5; deceler-ation time of early filling 140 ms), indicating high val-ues of mean left atrial pressure, and systolic pulmonaryhypertension was recorded (echocardiographic indirectestimation of pulmonary systolic pressure was 55mmHg, confirmed by right heart catheterization). Ac-cording to the serum levels of biochemical markers ofheart failure and the Doppler echocardiographic data adiagnosis of cardiogenic unilateral pulmonary edemacaused by acute worsening of mitral regurgitation washypothesized and therapeutic approach modified. Theclinical picture of the patient improved promptly afterintravenous administration of diuretics and vasodila-

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Figure 1. Chest X-ray performed on the day of acute dyspnea revealingthe presence of a right-sided pulmonary edema with a diffuse infiltrate.

Figure 2. Chest computed tomographic scan confirming the presence ofa unilateral interstitial infiltrate.

Figure 3. Echocardiographic examination showing severe mitral regur-gitation with a high velocity jet directed toward the right pulmonary vein.

tors; also the radiological picture of the lung edema re-solved (Figs. 4 and 5) together with a marked fall inBNP (506 pg/ml), and CA-125 (30 U/ml) values, as-sessed 24 hours after appropriate treatment.

Discussion

Asymmetric pulmonary edema is caused by localalteration of vascular homeostasis and it can be ex-plained by local imbalance of the Starling equation forincreased pulmonary venous pressure (cardiogenicedema), less frequently, by decreased oncotic pressure,by impaired lymphatic drainage or disruption of alveo-lar epithelial-endothelial integrity (non-cardiogenicedema)3.

Unilateral cardiogenic pulmonary edema is an un-usual clinical condition reported as a manifestation ofleft heart failure, mostly affecting the right lung. As de-scribed by Calenoff et al.4 pulmonary edema can beclassified as ipsilateral or contralateral with respect to

lung abnormalities. Ipsilateral pulmonary edema isusually due to acute alterations in the components ofthe alveolar-capillary complex in one lung only. Con-tralateral pulmonary edema refers to an edema occur-ring on the side opposite to the lung with a major per-fusion abnormality (Table I).

A D’Aloia et al - Right unilateral cardiogenic pulmonary edema

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Table I. Causes of ipsilateral and contralateral pulmonary ede-ma.

Ipsilateral cardiogenic pulmonary edema Left ventricular failure (acute mitral regurgitation) Unilateral veno-occlusive disease Extrinsic pulmonary venous compression (neoplasm)

Ipsilateral non-cardiogenic pulmonary edema Lung re-expansionAspiration Pneumonitis Surgical or traumatic injury (evacuation of hydrothorax orpneumothorax)

Contralateral pulmonary edema Unilateral pulmonary thromboembolismCongenital absence/hypoplasia of a pulmonary arterySwyer-James syndrome After pneumonectomy at the contralateral side Acute bronchial obstruction by a foreign body or tumor

Figure 4. Chest X-ray demonstrating the complete resolution of theright-sided pulmonary edema after medical treatment.

Figure 5. Chest computed tomographic scan confirming complete reso-lution of the interstitial infiltrate.

Unusual mechanisms have also been reported: leftto right shunts (for example, septal rupture)5, extrinsiccompression of a pulmonary vein by aneurysmal dila-tion of adjacent aorta or pulmonary artery6-9, acute mi-tral insufficiency secondary to paravalvular leak of aprosthetic mitral valve10-12, and gravitational edema.

Asymmetric pulmonary infiltrates of cardiac originare rare. In the majority of cases they are misdiagnosedbecause the likelihood of cardiac disease is not consid-ered and diagnosis of one of the more common causesof focal lung disease (such as pneumonitis, hemotho-rax, atelectasis or neoplasia) is often incorrectly made.

When pulmonary edema occurs in a patient with se-vere mitral regurgitation, it is usually cardiogenic. Rarecases of a regurgitant jet oriented toward the origin ofthe right pulmonary veins has been documented bytransesophageal echocardiography using color flowDoppler imaging in patients with asymmetric right-sided10-13 and, in one case, left pulmonary edema14.

The predilection of edema formation in the rightside is explained by the anatomy of the pulmonaryveins in relation to the mitral valve apparatus. Theplane of the mitral valve is inclined postero-superiorlyand to the right, and the regurgitant jet penetrates theorigin of the pulmonary vein in the right upper lobe.

Isolated or predominantly right upper lobe edema hasbeen reported in 9% of cases with severe mitral valve re-gurgitation4; these data underline the importance to con-sider acute mitral regurgitation for the differential diag-nosis of unilateral pulmonary involvement in patientswith known or suspected mitral valve disease.

In our patient the presence of a worsening mitral in-sufficiency (severe) was probably due to valve abnor-malities typical of hypertrophic cardiomyopathy, and tothe presence of systolic anterior movement of the mitralapparatus with variable degree of septal contact deter-mining an inconstant mild left ventricular outflow tractobstruction associated with severe left ventricular dias-tolic dysfunction. Finally, in this case the use ofDoppler echocardiographic examination and the deter-mination of biochemical markers of heart failure, suchas BNP and more recently CA-125, allowed both todemonstrate, respectively, the retrograde flow of bloodmainly into the right pulmonary veins as a pathogenet-ic mechanism of unilateral pulmonary involvement,and to confirm the cardiac origin of respiratory distress,although elevated levels of BNP may be associatedwith sepsis and pneumonia without primitive heart fail-ure15. In our case report the rapid disappearance of dys-pnea and of monolateral pulmonary infiltrates with car-diac marker reduction after diuretic therapy confirm thecardiac etiology of the clinical picture.

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