Kazuo Momma

Cardiovascular Malformations in the Conotruncal Anomaly Face Syndrome

In 1976, we reported a peculiar facial appearance [1] specifically related to conotruncal anomalies of the heart that was thus named “conotruncal anomaly face” (CTAF) and was characterized by lateral displacement of inner canthi, narrow palpebral fissures, flat nasal bridge, bloated eyelids, small mouth, hypoplastic mandibula, deformed earlobe, and nasal voice with velopharyngeal insufficiency [1–3].

Fetal and Neonatal Ductus Arteriosus Is Regulated with ATP-Sensitive Potassium Channel

The fetal patency and neonatal closure of the ductus arteriosus (DA) are regulated with oxygen and prostaglandins. The proposed oxygen sensors of fetal and neonatal DA include P450-endothelin and the Kv channel [1]. We hypothesized that the ATP-sensitive potassium channel (KATP channel) is another oxygen sensor [2].

Balloon Angioplasty of Pulmonary Artery Following Arterial Switch Operation for Complete Transposition of the Great Arteries

Twenty-eight patients with transposition of the great arteries underwent 39 balloon angioplasty procedures after the arterial switch operation. The mean age at dilation was 4.5 years (range, 0.7–10 years). The mean interval between operation and balloon dilation was 3.6 years. The criterion of successful dilation was an increase of 50% or greater in the predilation diameter or a decrease of 50% or greater in the predilation pressure gradient. The success rate of balloon angioplasty for pulmonary artery stenosis was 51%. The interval between operation and dilation in the successful dilation group was significantly less than that in the unsuccessful dilation group. In 13 patients who underwent balloon angioplasty less than 3.5 years after operation, balloon dilation was successful in 92%. A balloon-to-artery ratio of more than 2.3 was necessary to dilate the stenotic segment. There were no deaths, but one pulmonary artery rupture, which did not require surgical intervention, occurred. Aneurysmal dilation of the pulmonary artery was observed in 3 patients. These data indicate that although the success rate of balloon angioplasty for pulmonary artery stenosis after the arterial switch operation is modest (50%), balloon angioplasty can be the first therapeutic choice owing to the low complication rate and the potential benefit of the procedure. The success rate can be high if angioplasty is performed less than 3.5 years after operation and a balloon of adequate size is used.

Natural history and noninvasive of diagnosis of subpulmonic ventricular septal defect

Studies made on surgical as well as on autopsy cases of ventricular septal defect (VSD) at our Institute have indicated that after the (peri)membranous type of VSD, subpulmonic VSD (SPVSD; subarterial infundibular) is the most frequent type of VSD, in contrast to the frequency reported in the Caucasian series. The purpose of this presentation is to review the natural history of SPVSD based on cases experienced at the Heart Institute, Tokyo Women’s Medical College, and to discuss the specific features of various noninvasive diagnostic procedures identifying the subpulmonic location of VSD. The first part deals with the natural history. The natural history of SPVSD is unique in the following three aspects [1–3]: First, the aortic valve may prolapse into the SPVSD, resulting in aortic regurgitation [4–11]; second, aneurysm of the sinus of Valsalva may develop in association with the defect [11–13]; third, spontaneous closure rarely occurs.