Number 3 Brief Communications 815 disc lesions, such as hypertrophic cardiomyopathy, have frequently been reported. l-3 However, only one case of dilated cardiomyopathy has been described. 4 We report a patient with Noonan’s syndrome who had hypertrophic cardiomyopathy that progressed over 9 years to features suggesting dilated cardiomyopathy. A 22-year-old woman was readmitted to our hospital for further evaluation of cardiac function in November 1990. She was the firstborn child of a woman who was 26 years old at the time of her delivery. Her birth weight was 3600 gm. Her relatives had no histories indicating congenital malformation and they showed no abnormalities on the electrocardiogram. In 1968 she was admitted to the University Hospital because of a congenital diaphragmatic hernia (Bochdalek’s hernia) when cardiomegaly, congenital scoliosis, and a cleft palate were found. In 1981 she was first admitted to our department for further evaluation of left ventricular (LV) hypertrophy. Noonan’s syndrome was diagnosed on the basis of Turner’s syndrome-like phenotype in the presence of normal chromosomes. Hypertrophic cardiomyopathy was diagnosed by cardiac catheterization, echocardiography, and myocardial biopsy. When she was 19 years old, she began to experience progressive dyspnea. In 1990, she was readmitted to our hospital for further evaluation of cardiac function. She was 143.0 cm tall and weighed 47.0 kg. The chest x-ray films disclosed bilateral enlargement of the heart (cardiothoracic ratio= 58% in 1981 and 63I’C in 1990). Electrocardiography in 1981 revealed sinus rhythm and marked LV hypertrophy. Subsequently, the voltages of all leads decreased progressively. In 1990, a QS pattern of QRS morphology appeared in leads I and aVi,(Fig. 1). In 1981, an echocardiogram revealed marked LV hypertrophy without LV dilatation (LV enddiastolic dimension [LVDd]= 39 mm) and no systolic anterior motion of the mitral valve. Wall thickness of the intraventricular septum (IVS) was 26 mm and that of the LV posterior wall (LVPW) was 17 mm. In 1990, an echocardiogram showed mild dilatation of the LV cavity (LVDd= 51 mm) and disappearance of asymmetrical septal hypertrophy (IVS/LVPW= 14/15 mm) with a resultant decrease of IVS wall thickness from 26 to 14 mm. LV contraction decreased (LVDs/LVDd= 31/39 mm in 1981 and 47/51 mm in 1990) and the right ventricular (RV) cavity increased (Fig. 2). Cardiac catheterization was performed in 1981 and 1990. LV and RV end-diastolic pressure rose from 15 to 32 mm Hg and from 10 to 18 mm Hg, respectively. Coronary angiograms were normal. These hemodynamic findings confirmed that cardiac function had deteriorated over the previous 9 years. Lactic dehydrogenase (LDH) was elevated in both 1981 and 1990 (601 and 580 IU/L, respectively; normal limits= 146 to 382 IU/L), as were LDHi isoenzyme (36.2? and 40.05, respectively; normal= 20% to 30%), creatinine phosphokinase(CPK)(182 and 96 IU/L, respectively; normal= 0 to 52 IU/L), and creatinine phosphokinase isoenzyme (MB-CPK). Other blood chemistry and hormonal tests showed normal values.