The final therapy of 'end-stage heart failure' is orthotopic heart, lung or heart-lung transplantation. However, these options are not available for many patients worldwide. Therefore, novel therapeutical strategies are needed. Based on pathophysiological insights regarding (1) the long-term impact of an obstructive pulmonary outflow tract in neonates with congenitally corrected transposition o...

Zellweger syndrome (ZS) is a rare autosomal recessive inherited disorder within the spectrum of peroxisome biogenesis disorders. It is a progressive and fatal disorder with multiple congenital anomalies. There may be some challenges for anesthesiologists in patients with ZS. We report the anesthetic management of an infant with ZS undergoing closure of patent ductus arteriosus and pulmonary art...

SATO, T., ONOKI, H., KANO I., HORIUCHI BI, T., ISHITOYA, T., ABE, T., ISBIKAWA, S., TANAKA, S. and OKADA, Y . Balloon Atrial Septostomy in an Infant with Tricuspid Atresia. Tohoku J. exp. Med ., 1970, 101 (3), 281-288•\A fivemonth-old female with tricuspid atresia was successfully treated by Rashkind and Miller's balloon septostomy after banding of the pulmonary artery . tricuspid atresia; ball...

Following pneumonectomy (PNX), two separate mechanical forces act on the remaining lung: parenchymal stress caused by lung expansion, and microvascular distension and shear caused by increased perfusion. We previously showed that parenchymal stress and strain explain approximately one-half of overall compensation; the remainder was presumptively attributed to perfusion-related factors. In this ...

Follow-up cardiac catheterizations were carried out on 65 patients with transposition of the great arteries. Patent ductus arteriosus (PDA) was a significant problem in this group only when closure resulted in a precipitous drop in arterial oxygen saturation. Spontaneous closure of ventricular septal defects (VSD) occurred in approximately 20% of patients while natural or surgically created atr...

The total management of a group of 32 patients with transposition of the great arteries (TGA) is reported. All but one had required creation of an atrial septal defect in infancy. Eighteen patients had no associated cardiac defects, and physiologic correction was performed between 8 months and 9X years of age. The only death in this group occurred 9 months after operation. Eight of nine patient...

The lung was biopsied in 20 children with double inlet ventricle and pulmonary hypertension aged 2 months to 14 years. Eleven patients had two patent atrioventricular valves, three atresia of the right valve, and six hypoplasia of the left valve. Severe pulmonary arterial medial hypertrophy occurred in the nine children less than 1 year of age. The findings did not suggest a period of normality...

Pulmonary artery banding (PAB) is a method for reducing pulmonary artery blood flow and pressure by tying a surgical band around the main pulmonary artery (MPA). Originally, the procedure was primarily performed to palliate intracardiac left to right shunts for ventricular septal defects (VSDs). Currently, the use of PAB has expanded to allow it to be used in ventricular training, prior to tota...

BACKGROUND Increased mortality in patients with chronic pulmonary hypertension has been associated with elevated right atrial (RA) pressure. However, little is known about the effects of chronic right ventricular (RV) pressure overload on RA and RV dynamics or the adaptive response of the right atrium to maintain RV filling. METHODS AND RESULTS In 7 dogs, RA and RV pressure and volume (conduc...