Toshihiro Ino

Application of Percutaneous Transluminal Coronary Angioplasty to Coronary Arterial Stenosis in Kawasaki Disease

BACKGROUND Percutaneous transluminal coronary angioplasty (PTCA) has rarely been performed on patients with coronary lesions that result from Kawasaki disease. In this study, we retrospectively evaluated the effectiveness of PTCA in five patients with coronary arterial stenosis that resulted from Kawasaki disease and reviewed previous reports for possible indicators of PTCA effectiveness. METHODS AND RESULTS Five patients, ranging in age from 2 to 16 years (median 8 years) underwent conventional PTCA for localized stenosis. The lesion targeted for PTCA was located in the middle right coronary artery of three patients and in the left anterior descending artery in two patients. In four of the five patients, PTCA was angiographically effective, with stenosis rates improving from 84 +/- 10% to 33 +/- 11% (P<.05). When the previously reported cases of six similar patients were taken into consideration, the only predictor of successful PTCA seemed to be the time elapsed between the onset of Kawasaki disease and performance of this procedure. CONCLUSIONS In cases in which patients show significant localized stenosis as a result of Kawasaki disease, PTCA should be attempted within 6 to 8 years of the onset of the disease. Additionally, intravascular ultrasound imaging was found to be a useful tool for evaluating internal morphology before and after PTCA. In older patients with coronary calcification, other alternatives to PTCA, such as the use of a rotablator or an atherectomy catheter, should be considered.

Thrombolytic therapy for femoral artery thrombosis after pediatric cardiac catheterization

Femoral artery thrombosis remains a well-known complication after cardiac catheterization. A study was undertaken to assess the efficacy of thrombolytic therapy for this complication. A total of 526 consecutive infants and children were prospectively evaluated after cardiac catheterization, and the medical charts of 42 patients who required femoral artery thrombectomy between 1975 and 1985 were reviewed. In the prospective study, patients were given a bolus injection of heparin, 150 U/kg, at the time the artery was entered. Patients with persistently absent or diminished pulse 2 hours after catheterization received a second bolus injection of 50 U/kg followed by an infusion of 20 U/kg/hr heparin for a maximum of 48 hours. If the affected leg pulse was absent or reduced and the systolic Doppler blood pressure was less than two thirds that of the unaffected leg, thrombolytic therapy was begun. In the 42 patients with surgical thrombectomy, there were no serious complications of surgery. Forty-five of the 526 patients (8.6%) had a decreased or absent pulse after catheterization. Of these 45 patients, 32 (71.1%) improved with systemic heparinization only. Thirteen patients (28.9%) had a persistently absent pedal pulse suggesting femoral artery thrombosis, despite continuous heparinization. Eleven patients were successfully treated with thrombolytic therapy and two required surgical thrombectomy. Intraarterial balloon dilatation procedures were performed in 8 of these 13 patients. Prothrombin time was prolonged (11.5 +/- 1.06 to 52.3 +/- 40.4 seconds; p less than 0.025) and fibrinogen levels were significantly reduced (2.25 +/- 0.79 to 1.52 +/- 0.52 gm/dl; p less than 0.01) during therapy. There were no serious complications, although four patients bled from the groin entry site.(ABSTRACT TRUNCATED AT 250 WORDS)

Natural history and prognostic risk factors in endocardial fibroelastosis

A retrospective study was made of 52 patients (16 men, 36 women) with endocardial fibroelastosis diagnosed by strict clinical criteria and confirmed histologically in 18 (35%). Clinical and hemodynamic manifestations at presentation were reviewed from the clinical record. The follow-up period averaged 47 months (range 1 day to 228 months). Actuarial survival rates were 93% at 6 months, 83% at 1 year and 77% at 4 years. Clinical and hemodynamic manifestation included onset at less than 1 year of age (89%), respiratory distress (71%), cardiomegaly on chest roentgenogram (99%), left ventricular hypertrophy with ST-T-wave changes on the electrocardiogram (97%) and reduced contractility with dilatation of the left ventricle (100%). Prognostic risk factors were evaluated comparing 13 patients who died (group 1) with 16 patients who survived greater than 4 years (group 2). Only cardiac index (2.8 +/- 0.8 vs 3.5 +/- 0.5 liter/min/m2) and ejection fraction (18 +/- 12 vs 33 +/- 21%) measured at catheterization were significantly reduced in group 1 compared with group 2 (p less than 0.005 and p less than 0.01, respectively). Careful observation and appropriate management are recommended in all patients, although low ejection fraction and cardiac output at presentation are predictive of poor outcome and support other therapeutic alternatives.

Pentoxifylline and intravenous gamma globulin combination therapy for acute Kawasaki disease

We compared the efficacy of oral administration of pentoxifylline (PTX) and intravenous infusions of gamma globulin (IVGG) combination therapy with that of IVGG in reducing the frequency of coronary-artery lesions (CAL) in children with Kawasaki disease (KD), in a randomized trial. All patients with KD received acetylsalicylic acid (30 mg/kg per day), until the 30th day, after the onset of fever, followed by daily acetylsalicylic acid at a dose of 3-5 mg/kg per day there-after, and intravenous IVGG, 200 mg/kg per day, for 5 consecutive days. In addition, patients randomly assigned to PTX and IVGG combination therapy groups received oral PTX at a dosage of 10 mg/kg per day (low-dose) or 20 mg/kg per day (high-dose), in three divided doses until the 30th day. Patients with KD were all free from CAL prior to treatment. We assessed the presence of CAL by two-dimensional echocardiography which was also done prior to treatment and then twice a week after hospital admission. We detected CAL in 3 of 18 patients (16.7%) in the IVGG therapy group, as compared with 2 of 18 patients (11.1%) in the low-dose PTX and IVGG combination therapy group. There were no significant differences between the two groups. In the next study, we detected CAL in 3 of 21 patients (14.3%) in the IVGG therapy group, as compared with none of 22 patients (0%) in the high-dose PTX and IVGG combination therapy group (χ2 = 6.4, P < 0.02). No adverse side-effects were observed in 79 patients with KD.

Apoptosis as a Possible Cause of Wall Thinning in End-Stage Hypertrophic Cardiomyopathy

A 15-year-old boy with hypertrophic cardiomyopathy died of congestive heart failure with progressive left ventricular wall thinning with poor systolic function. Microscopic examination revealed patchy fibrosis in the ventricular myocardium with wall thinning, and immunohistochemical evaluation of apoptosis showed apoptotic cells and bodies in the destroyed myocytes along the border between the fibrotic area and myofibril.

Cardiac manifestations in disorders of fat and carnitine metabolism in infancy.

The prognosis of patients with cardiomyopathy associated with hypocarnitinemia is uncertain. Cardiac hemodynamics, histologic findings and response to oral L-carnitine therapy were retrospectively evaluated in 11 children with cardiomyopathy associated with abnormal carnitine metabolism. Three had systemic carnitine deficiency, two familial hypocarnitinemia with neutropenia, three transient neonatal hypocarnitinemia and three a carnitine insufficiency syndrome. Six had a hypertrophic and five a dilated cardiomyopathy. Hypotonia was present in seven (64%). The cardiothoracic ratio was greater than 0.60 in eight (73%). The most frequent abnormality on the electrocardiogram was ST-T wave inversion in the left precordial leads with various degrees of left ventricular hypertrophy. Echocardiographically, two patients with hypertrophic cardiomyopathy had decreased left ventricular function and two patients with dilated cardiomyopathy had increased thickness of the left ventricular wall. Histologic evaluation (two autopsies and one endomyocardial biopsy) revealed striking lipid accumulation within hypertrophied myocytes. Six of eight patients on carnitine replacement therapy had improvement echocardiographically during a 3 month to 2 year follow-up period. In summary, both hypertrophic and dilated cardiomyopathy can result from abnormal carnitine metabolism. The determination of plasma carnitine concentrations and fatty acid metabolism by-products should be performed in all patients with either form of cardiomyopathy of unknown etiology because carnitine supplementation may lead to improvement.

Age Dependency of Stiffness of the Abdominal Aorta and the Mechanical Properties of the Aorta in Kawasaki Disease in Children

Abstract. Measuring aortic distensibility has been shown to be useful in adults as a noninvasive method in the early detection of atherosclerosis. This study had two purposes: to assess the stiffness of the abdominal aorta by using two-dimensional echocardiography (2DE) in healthy neonates, children, and adults and to assess aortic distensibility in children with Kawasaki disease in acute and subacute phases. The study comprised 168 healthy subjects and 40 patients with Kawasaki disease. We recorded systolic (Ps) and diastolic (Pd) blood pressure and measured aortic diameter (Dd) at both minimum diastolic pressure and maximum systolic expansion (Ds) by 2DE. These measurements were used to determine (1) aortic strain (S) = (Ds−Dd)/Dd, (2) pressure strain elastic modulus (Ep) = (Ps−Pd)/S, and (3) normalized Ep (Ep*) =Ep/Pd. Significant correlations were found between S and age, Ep and age, and Ep* and age. In Kawasaki disease, Ep and Ep* showed negative correlations to day after onset. The aorta was less distensible in infants, became soft in 12- to 16-year-olds, and then stiffened with increasing age among normal subjects. In Kawasaki disease, aortic stiffness was high at the acute phase and normal at the subacute phase. These tendencies may be related to the biological characteristics of smooth muscle cells.

Dilated cardiomyopathy with neutropenia, short stature, and abnormal carnitine metabolism.

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Dilatation Mechanism of Balloon Angioplasty in Children: Assessment by Angiography and Intravascular Ultrasound

AbstractPurpose: Little information is available about the dilatation mechanism in children. This prospective study aimed to (1) evaluate the dilatation mechanism of balloon angioplasty in children with arterial stenosis, and (2) compare the morphological changes seen by intravascular ultrasound (IVUS) and angiography. Methods: Twenty consecutive patients, who had undergone a total of 23 procedures, were examined before and immediately after balloon angioplasty with a 4.3 Fr, 30 MHz rotational tip IVUS system. The lesions for IVUS study had resulted from coarctation of the aorta in six patients, pulmonary arterial stenosis in five, Blalock-Taussig shunt stenosis in three, subclavian artery stenosis in two, renal artery stenosis in two, coronary artery stenosis in one and ductus arteriosus in one. Results: Four distinctive morphological types were identified: type I with arterial stretching, type IIa with superficial tearing, type IIb with deep intimal-medial tearing, type III with flap formation, and type IV with dissection. The diameter of the narrowest site before and after balloon angioplasty increased significantly from 2.1 ± 1.4 mm to 4.6 ± 3.4 mm (p < 0.001). Eighteen of the 23 angioplasty procedures (78%) were considered to be successful, with a dilatation ratio of more than 50%. In most patients with successful dilatation, non-stretch mechanisms such as tearing, flap formation, or dissection were found. The positive percent (70%) of non-stretch mechanisms seen by IVUS was significantly higher than the positive findings (39%) by angiography (Χ2= 6.47, p < 0.02). Conclusions: Non-stretch morphology of the arterial wall may be a common mechanism of dilatation after balloon angioplasty in children with arterial stenosis. IVUS is a useful modality for evaluating the effectiveness of balloon angioplasty and the mechanism of dilatation in individual cases.

Progressive vascular lesions in Williams-Beuren syndrome

SummaryWe report two patients with Williams-Beuren syndrome. The first patient showed no evidence of coarctation of the aorta at the first examination. Seven years later, she developed coarctation of the aorta. In the second patient, we found the progression of renal artery stenosis by serial angiography. We report that vascular lesions may be progressive in Williams-Beuren syndrome.