The Lutembacher's syndrome is one of the very rare diseases. A few reports concerned have been presented in Japan, and there were only two cases that were diagnosed as Lutembacher's syndrome with cardiac catheterization.We report here a case in which we suspected the diagnosis of Lutembacher's syndrome, first by means of routine clinical examinations and finally confirmed the diagnosis by cardiac catheterization.The patient was a 62 year-old, married female. Chief complaints were palpitation and edema of the whole body. Her family and personal history were not significant. When she was a child her family physician told her that her heart was "weak", but she had 9 deliverys and no trouble with her daily life.The physical examination revealed; An enlargement of the area of cardiac dullness to both sides, and the cardiac pulsation was palpated over a large area near the sixth intercostal space and the center of this area was 3 cm left of the midclavicular line. Slight systolic, and rumbling diastolic murmurs were heard at the cardiac apex. There were also a strong systolic murmur and an accentuation and separation of the second sound at the auscultatory area of the pulmonary ostium (Fig. 1, Fig. 5).Arterial blood pressure was 140/80 mmHg. Venous pressure was 250 mm H2O. The congested liver was palpated and signs of congestive kidney were present in the urine. On a X-ray of the heart (Fig. 2), we observed a hill-formed enlargement of the second left arch, enlagement of the third and forth left arches and the second right arch. On an oblique X-ray of the heart (Fig. 3), we saw the enlarged shadow of the left auricule and pulmonary artery. Hilar dancing was seen with the fluorescopic examination. On the electrocardiogram we found auricular fibrillation with premature ventriclar beats, a high degree of right ventriclar hypertrophy and right axis deviation (Fig. 4).From the above findings we suspected the Lutembacher's syndrome and the cardiac catheterization was performed for its confirmation.The results of cardiac catheterization (Tab. 1, Fig 6) : The O2-content of the blood in the right auricule (11.8%) was 3% higher than that of the vena cava cranialis and caudalis (7.9∼9.6%). This fact shows the presence of a left-to-right shunt in the area of the right auricule.The calculated blood flows were as follows : The flow of the systemic circulation was 5.3 l/m, that of the pulmonary circulation was 12.6 l/m and that of the interatrial shunt was 7.3 l/m. Consequently, the blood flow of the pulmonary circulation was more than twice as that of the systemic circulation.The intercardiac pressures were as follows : In the right auricule the maximum, the minimum and the mean values were 29, 14 and 20 mmHg ; in the right ventricle 93, 0 and 43 mmHG ; and in the pulmonary artery 80, 32 and 47 mmHg. The values of the pulmonary arterial wedge pressure were 37, 21 and 29 mmHg. Each of these values were remarkably higher than normal.Diagnosis : In order to interpret the meaning of these values we compared the values of our case with those found in the literature (Tab. 2).Three types of left-to-right shunts in the area of the right auricule, which are not complicated with mitral disturbances (II, a. b. c.), can be excluded, because there is no raise of the right auricular pressure, while in our case the right auricular pressure is remarkably elevated. In mitralstenosis which is not complicated with an interatrial comunication (III), the right auricular pressure is not raised as high as in our case. In cases of left-to-right shunt, in the area of the right auricule, other than interatrial communications, which are complicated with mitral disturbances (IV, a. b.), the right auricular pressure would not be elevated, hence those cases should be excluded.
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Nov 29, 2022
Ahmed Serwa 
Open Access
