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Anatol J Clin Investig 2008:2(2):86-87 RHEUMATIC STENOSES OF ALL FOUR CARDIAC VALVES: A CASE REPORT TÜM KALP KAPAKLARININ ROMATİZMAL STENOTİK TUTULUMU: VAKA RAPORU Ednan BAYRAM1, Hasan KOCATÜRK2, Mehmet Cengiz ÇOLAK3, Murat MERİÇ1 1 2 3 Numune Hastanesi, Kardiyoloji Servisi, Erzurum, Türkiye Şifa Hastanesi, Kardiyoloji Servisi, Erzurum, Türkiye Şifa Hastanesi,kardiyovasküler Cerrahi Servisi, Erzurum, Türkiye Abstract Affection of all four valves is extremely uncommon feature of rheumatic heart disease. The few reports in the literature of patients with rheumatic lesions of all four heart valves correspond to cases diagnosed at autopsy or by cardiac catheterization, but reports of echocardiographic diagnosis are much rarer. Echocardiography is useful for confirming clinical findings and allows assessment of the severity of valvular stenosis and regurgitation. (Anatol J Clin Investig 2008:2(2);86-87). Özet Romatizmal kalp hastalıklarında kalbin dört kapağının da birlikte tutulması oldukça nadir bir durumdur. Bu konuda literatürde çok az sayıda vaka vardır. Bu vakaların çoğunluğun tanısı otopsi veya kardiyak kateterizasyonla konulmuştur, ancak ekokardiyografik olarak tanı konulan sayı oldukça azdır. Ekokardiografi kapakların yetersizliği / darlığını ve ciddiyetini saptayan klinik bulgularla uyumlu bir araçtır. (Anatol J Clin Investig 2008:2(2);86-87). Introduction Quadrivalvular rheumatic damage is more rare[1]. The few reports are available in the literature describing of patients with rheumatic lesions of all four heart valves [2,3]. To our knowledge, only 12 cases have been reported in the literature [110]. Echocardiography provides crucial information in the evaluation of a patient with suspected significant heart valve dysfunction, and is can contribute to earlier diagnosis. Also there are a few reports of echocardiographic diagnosis of stenotic involvement of all four cardiac valves [4]. Case report A 41 year old man presented with exertional dyspnea, palpitations and fatigue of 8-year duration. He had New York Heart Association class IV symptoms. Clinical findings suggested severe stenosis of the mitral, aortic and tricuspid valves with a history of rheumatic fever in childhood. Chest X-ray radiography showed gross cardiomegaly and was consistent with pulmonary arterial hypertension. The electrocardiogram suggested severe left atrial overload and right ventricular hypertrophy and was in sinus rhythm. There were opening snaps in the apex and in the lower left sternal border. A grade 4 ejection murmur in the aortic area conducted to carotids. Liver was palpable 3 -cm below the right costal margin. Transthoracic echocardiography revealed severe aortic, mitral, and tricuspid valve stenoses and calcium deposition on the tips of the pulmonary valve with doming. The mitral and aortic valve areas were 1 and 0.9 cm2, respectively. The mean gradient across the mitral and tricuspid Ednan BAYRAM Numune Hastanesi, Kardiyoloji Servisi, Erzurum, Türkiye E-mail: [email protected] valve were 16 and 14 mm Hg, respectively. The peak systolic gradient was 105 mm Hg across the aortic valve and 57 mm Hg across the pulmonary valve. There was minimal aortic and pulmonary regurgitation and moderate tricuspid regurgitation but no regurgitation over the mitral valve. Discussion Affection of all four valves is an uncommon feature of rheumatic heart disease, with stenosis in all valves being still rarer [1]. The few reports in the literature of patients with rheumatic lesions of all four heart valves correspond to cases diagnosed at autopsy or by cardiac catheterization, but reports of echocardiographic diagnosis are much rarer [2-4]. Before the development of echocardiography, cardiac disease was diagnosed with a loud heart murmur and clinical signs of congestive heart failure (coughing, edema, venous distention, jugular pulsations) detected on physical examination. Earlier, pulmonary valve involvement was diagnosed only at surgey [5]. This case highlights the importance of preoperative diagnosis of pulmonary valve involvement. The late recognition of pulmonary stenosis on the operating table extended the bypass time, which probably contributed to the fatal complication of disseminated intravascular coagulation [5]. There are a few reports of echocardiographic diagnosis of rheumatic lesions of all four heart valves [4]. Preoperative echocardiographic diagnosis the involvement of all four cardiac valves and successful surgical management has also been reported [6]. Echocardiography is useful for confirming clinical findings and allows assessment of the severity of Rheumatic stenoses of all four cardiac valves: a case report valvular stenosis and regurgitation [7,8], and can contribute to earlier diagnosis. Also operator awareness regarding possible involvement of all four valves is essential for appropriate diagnosis [9]. It is possible that by the time echocardiography came into common use, the incidence as well as the severity of rheumatic heart disease had already been drastically reduced. This could explain the rarity of echocardiographic detection of quadrivalvular rheumatic involvement, which seems to be the case even in developing countries [10]. We reported in this patient with quadrivalvar involvement by rheumatic disease, diagnosed preoperatively by echocardiography, which underwent successful surgical correction. Figure 1. Transthoracic echocardiogram showing thickened and doming mitralvalve (A) and thickened aorta valve (B). Figure 2. Transthoracic echocardiogram showing thickened and doming tricuspid valve (A) and thickened pulmonary valve (B). References. 1. 2. Quadrivalvular heart disease. In: Hurst JW, ed. The heart – update III. New York: Mc Graw-Hill, 1980; 120-129. Gialloreto o, Aerichide N, Allard PP. Stenotic involvement of all four heart valves: report of three cases. Am J Cardiol 1961;7: 865-873. 3. Roberts WC, Virmani R. Aschoff bodies at necropsy in valvular heart disease: evidence from an analysis of 543 patients over 14 years of age that rheumatic heart disease at least anatomically is a disease of the mitral valve. Circulation 1978; 57:803-807. 4. Bandin MA, Vargas-Barron J, Keirns C, Romero-Cardenas A, Villegas M, Buendia A. Echocardiographic diagnosis of rheumatic cardiopathy affecting all four cardiac valves. Am Heart J 1990;120:1004–1007. 5. Kumar AS, Iyer KS, Chopra P. Quadrivalvular heart disease. Int J Cardiol 1985;57:66-69. 6. Kumar N, Rasheed K, Gallo R, Al-Halees Z, Duran CM. Rheumatic involvement of all four heart valves--preoperative echocardiographic diagnosis and successful surgical management.Eur J Cardiothorac Surg. 1995;9:713-4. 7. Vasan RS, Shrivastava S, Vijayakumar M, Narang R, Lister BC, Narula J. Echocardiographic evaluation of patients with acute rheumatic fever and rheumatic carditis.Circulation.1996;94:73-82. 8. Narula J, Chandrasekhar Y, Rahimtoola S. Diagnosis of active rheumatic carditis:The echoes of change.Circulation 1999;100:1576-1581 9. Jai Shankar K, Jaiswal PK, Cherian KM. Rheumatic involvement of all four cardiac valves. Heart.2005;91:50. 10. Krishnamoorthy KM. Images in cardiovascular medicine. Rheumatic stenosis of all four valves. Tex Heart Inst J. 2002;29(3):224-5. 87