Masazumi Iwahara

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.

Clinical features of aortic arch anomaly with malalignment ventricular septal defect.

The clinical features and outcome after various surgical procedures on 9 patients with coarctation or interruption of the aortic arch and malalignment ventricular septal defect (group 1) were compared with those of 9 patients with the arch anomaly without malalignment ventricular septal defect (group 2). Cardiomegaly and metabolic acidosis were prominent in group 1. Five of the 9 patients in group 1 died in the immediate postoperative period (56% mortality), but no operative deaths occurred among 8 patients in group 2 (p less than 0.01). The ratio of left ventricular outflow tract to ascending aortic diameter was 0.59 +/- 0.09 in group 1 and 1.03 +/- 0.11 in group 2 (p less than 0.01). Three of 4 patients with a ratio of less than 0.6 died, but no operative deaths occurred among the 6 patients who had a palliative operation and in whom the ratio was more than 0.6. These data suggest that left ventricular outflow tract obstruction is critical when the ratio of left ventricular outflow tract to ascending aortic diameter is 0.6 or less. The presence of severe left ventricular outflow tract obstruction necessitates modification of the present surgical strategy.

Plasma endothelin concentration: Relation with vascular resistance and comparison before and after balloon dilatation procedures

Endothelin (ET) is a potent vasoconstrictor peptide with an as yet uncertain physiological role in cardiovascular disease. We measured blood plasma ET concentrations using a recently developed radioimmunoassay and analysed the relations between ET concentration, systemic arterial pressure and systemic vascular resistance. In addition, ET levels before and after percutaneous balloon valvuloplasty and angioplasty were measured. Fifty-one patients were studied: (1) 13 patients with small left-to-right shunting or Kawasaki heart disease (age ranged from 4 to 144 months); (2) 10 patients who had undergone ballon valvuloplasty or angioplasty (age ranged from 1 to 233 months) and (3) 28 healthy infants and children (age ranged from 3 to 152 months). Systemic vascular resistance was calculated by the formula (mean aortic pressure — mean right atrial pressure) X 80/cardiac output (dyne·sec·cm−5). Plasma ET concentrations in healthy children less than 2 years were significantly higher than those over 2 years (2.48±0.62 vs 1.31±0.53 pg/ml). In eight patients in groups 1 and 2, plasma ET concentration in the pulmonary artery (2.00±0.43 pg/ml) was significantly lower than that in the femoral vein (2.39±0.69 pg/ml) and aorta (2.23±0.59 pg/ml), suggesting ET secretion derived from endothelial cells in peripheral pulmonary vessels. There was a significant positive correlation between ET concentrations in the femoral vein and systemic vascular resistance (r=0.55,p<0.05). After balloon dilatation ET concentration rose from 2.15±0.82pg/ml to 2.61±1.38 pg/ml. These results suggest that ET may be a stress-induced hormone which is secreted by the transient hypotension following percutaneous balloon dilatation and which regulates peripheral vascular tonus.

Successful balloon mitral commissurotomy in a small child: Use of small Inoue balloon catheter

A 15-month-old girl weighing 5.8 kg, in whom congestive heart failure developed due to congenital mitral stenosis, was successfully treated by percutaneous transluminal mitral commissurotomy using a small Inoue balloon catheter. Percutaneous transluminal mitral commissurotomy using a small Inoue balloon catheter may be a first-choice treatment for small children with congenital mitral stenosis.

Aortocoronary bypass surgery for Kawasaki disease

SummaryA case of Kawasaki disease with precordial pain in a 6-year-old boy is described. Coronary arteriography revealed large aneurysms with subtotal obstruction of the left anterior descending artery and long-segment narrowings of the right coronary artery. The left ventriculogram revealed an old myocardial infarction of the inferoposterior wall of the left ventricle. The patient was given aortocoronary bypasses using both a left internal mammary artery and a fresh saphenous vein graft to the left anterior descending artery. Serial angiographic study, performed 6 weeks after the operation, revealed these grafts were both patent. Using the two techniques together may have the potential benefit of the saphenous vein bypass graft functioning as a temporary measure to ensure survival with the hope that the internal mammary artery supply will continue to grow.

Advanced atrioventricular block in a neonate with lupus erythematosus and anti-SS-A antibodies.

SummaryRecent studies suggested the involvement of anti-SS-A and anti-SS-B antibodies in the pathogenesis of neonatal lupus erythematosus and congenital heart block. We here describe a female neonate with lupus erythematosus, advanced atrioventricular block, and a positive test for anti-SS-A antibodies. The findings of His-bundle recordings are presented.

Balloon occlusion aortography

We review the validity of balloon occlusion aortography (BOA) on the basis of our personal experience with 18 patients with congenital heart disease (mean weight 4.55 g, including 8 neonates). Four of the 18 patients underwent aortic arch angiography using balloon occlusion of the descending aorta. Pulmonary angiography was also performed in 9 patients via a patent ductus arteriosus and in 3 patients via a Blalock-Taussig shunt. The remaining 2 patients underwent coronary arteriography by balloon occlusion of the ascending aorta. The information obtained was satisfactory in 17 of the 18 patients. However, in one patient with a double-outlet right ventricle and pulmonary stenosis, the pulmonary arteries were not clearly visualized because of dominant antegrade flow from the right ventricle. BOA is a safe and useful procedure which can be used to image the aortic arch, pulmonary artery, and coronary arteries in infants with congenital heart diseases. In children over 3 years of age, however, the balloon may not be able to occlude the appropriate site of the aorta, so selective angiography is required to obtain precise information.

Lateral view of chest x‐ray in long‐term follow‐up of Kawasaki disease as a useful screening method for the cardiac sequela

Kawasaki disease (mucocutaneous lymph node syndrome) is thought to be one of the vaculitis syndromes such as anaphylactoid purpura, polyarteritis nodosa or Takayasu disease, although the etiology is still not known. The diagnosis is based on the typical constellation of signs and symptoms. Of the variety of complications associated with Kawasaki disease, cardiac complications as manifested by coronary artery aneurysms, coronary arteritis and carditis are probably the most distinctive and most feared. These, especially coronary aneurysm, may result in rupture or myocardial infarction followed by sudden death in 1% to 2% of the patients. Cross-sectional echocardiography and selective coronary arteriography are very useful investigations to detect the coronary aneurysm. The purpose of this report is to describe the importance of lateral view of chest x-ray as a useful screening for coronary aneurysm which often has calcification after longstanding process following Kawasaki disease at the outpatient clinic of general practice.