[Repair of total anomalous pulmonary venous return below the age of three months without aortic cross-clamping].
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Biomedical subjects
Publications and source records attributed to M Aoshima.
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A yeast gene termed YKR coding for a putative protein kinase was isolated by using the cloned cDNA for rabbit protein kinase C as a hybridization probe. The encoded protein (YKR), composed of 380 amino acid residues, shows extensive sequence homology to serine/threonine-specific protein kinases from various species in the approx. 320 C-terminal amino acid residues, strongly suggesting that YKR is endowed with a protein kinase activity. The observed homologies to the cdc25 suppressing protein kinase from yeast, the catalytic subunit of mammalian cAMP-dependent protein kinase, and mammalian protein kinase C were 76, 48 and 37%, respectively. Gene replacement experiments showed that YKR itself is not essential for cell proliferation.
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Anatomical repair was successfully performed in an infant with transposition of the great arteries and intact ventricular septum who had previously received a Blalock-Taussig shunt. In this patient, creation of an aortopulmonary anastomosis induced a well-developed left ventricle with concomitant adequately increased pressure and mass. Beneficial aortopulmonary shunt that precedes the anatomical repair in selected patients with simple transposition is discussed.
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In the treatment of cyanotic heart diseases in neonates who are ductus dependent, the small size of the pulmonary artery and/or subclavian artery often precludes construction of a sufficient Blalock-Taussig shunt. To avoid an emergency operation and to obtain pulmonary and subclavian arteries of adequate size (3 mm), a low dose of prostaglandin E1 was given to 16 neonates for a mean of 47 days (range 26 to 65 days), and a modified Blalock-Taussig shunt operation was performed in neonates whose mean age was 50 days. The pulmonary artery grew significantly (mean 4.5 mm) and the mean diameter of the subclavian artery was 3 mm, which was large enough for a good shunt at the time of operation. There were two late deaths and one operative death. All surviving patients shunts at the mean follow-up period of 22 months. The shunt was constructed on the same side as the ductus arteriosus so that the ductus could be ligated at the same operation in selected cases; the ductus may not close after discontinuation of prostaglandin E1 infusion and may cause uncontrollable congestive heart failure because of excessive pulmonary blood flow, particularly in patients with a univentricular heart. The ductus, dissected in nine patients, was enlarged and elongated in all and was ligated in four patients without complication.
The effect of preoperative left ventricular end-diastolic volume on hemodynamics after repair and the safety limit of left ventricular end-diastolic volume for total correction of tetralogy of Fallot were studied. Preoperative left ventricular volume was determined in 38 patients according to the area-length method from biplane cineangiocardiograms. The mean left ventricular end-diastolic volume of the 38 patients was 83% +/- 23% of normal. The left ventricular end-diastolic volume of 20 corrected patients (90% +/- 22% of normal) was significantly larger (p less than 0.05) than that of 18 with a systemic-pulmonary shunt (75% +/- 22% of normal). In the corrected patients, the total amount of dopamine required in the postoperative period showed an excellent inverse exponential correlation with the preoperative left ventricular end-diastolic volume (r = -0.826); it showed a poor inverse correlation with the ratio of pulmonary artery to aortic diameter (r = -0.587) and with myocardial ischemic time (r = -0.487); and it showed no correlation with postoperative right to left ventricular systolic pressure ratio (determined at the time of the chest closure) and residual right ventricular-pulmonary arterial pressure gradients. The patients with a left ventricular end-diastolic volume under 70% of normal had severe low-output syndrome after total correction. We recommend a left ventricular end-diastolic volume of 60% of normal as a safety limit for total correction in patients under 2 years of age. In patients over 2 years of age, the safety limit may be larger because there are more collaterals with increasing age.