Right-sided pulmonary aplasia: longitudinal lung function studies in two cases and comparison to results from term healthy neonates.

Agenesis of the right lung was diagnosed prenatally in two neonates born at 36 and 37 weeks, respectively. Computed tomographic scans and magnetic resonance imaging indicated that both cases had a Type 2 pulmonary agenesis, which was confirmed later by bronchoscopy. Both patients were clinically stable during the neonatal period. Serial pulmonary function tests revealed a decrease in specific respiratory system compliance (sCrs) in both neonates and a marked discrepancy between functional residual capacity measured by the nitrogen washout technique (FRCN2) and by plethysmography (FRCpleth) on follow-up. Early decrease of respiratory system compliance (Crs) and increase of respiratory system resistance (Rrs) in one infant preceded the onset of tracheal stenosis, which remained asymptomatic until the age of 8 weeks, when the infant developed acute respiratory failure requiring intubation and mechanical ventilation with high airway pressures. Aortopexy, implantation of a tissue expander into the right hemithorax, and laser ablation of fibrotic tissue at the site of tracheal stenosis were performed to achieve successful extubation. The second infant remained asymptomatic. Values for lung mechanics and volumes for both infants with pulmonary aplasia were as follows: Crs, 3.43 and 10.60 mL x kP(-1) x kg(-1); sCrs, 0.23 and 1.28 kpa(-1); Rrs, 11.1 and 7.4 kpa x s x L(-1); FRCN2, 14.9 and 10.2 mL x kg(-1); FRCpleth, 28.2 and 25.8 mL x kg(-1); FRCN2: FRCpleth ratio, 0.56 and 0.54 for patients 1 and 2, respectively. These values differed considerably from results of a control group of nine term healthy neonates (Crs, 10.0+/-1.8 mL x kPa(-1) x kg(-1); sCrs, 0.43+/-0.08 kpa(-1); Rrs, 5.10+/-0.55 kpa x s x L(-1); FRCN2, 24.0+/-2.5 mL x kg(-1); FRCpleth, 31.1+/-6.0 mL x kg(-1); FRCN2:FRCpleth ratio, 0.78+/-0.10). In conclusion, serial assessment of lung mechanics and pulmonary gas volumes detects airway obstruction early in neonates with unilateral lung agenesis. Bronchoscopy is recommended. Along with conventional surgical procedures, an expandable implant may improve management or prevent respiratory failure in selected cases.

Surgical removal of atrial septal defect occlusion system-devices.

OBJECTIVE: Since 1976 a number of similar transvenous atrial septal defect occlusion systems have been developed and applied to prevent surgical intervention with extracorporeal circulation and the complications involved. One of them is the so called atrial septal defect occlusion system (ASDOS-device; Dr Ing Osypka, Germany). METHOD AND RESULTS: In our institution, eight ASDOS-devices were implanted in adults during the last year. In six individual cases, these devices were explanted because of thrombosis, residual shunts, deformations of the umbrella, disconnection of the system and atrial perforation with hemopericard. This occured 17-380 days after the implant. The surgical removal was uncomplicated in all cases. CONCLUSIONS: Because the complication rate of the ASDOS-device was unacceptably high, surgical repair was preferred.

Pulmonary agenesis in a newborn: implantation of tissue expander to prevent a mediastinal shift.

Unilateral pulmonary agenesis is a rare congenital anomaly whose aetiology is not completely known. Vitamin A deficiency, viral agents, or genetic factors are discussed. The incidence was estimated at 1 in 10,000-15,000 autopsies by several authors. Associated malformations, mainly affecting the cardiovascular, gastrointestinal, and musculoskeletal system influence the prognosis of patients as does the location of the missing lung. We report the implantation of a tissue-expander in a three-months-old infant suffering from a right-sided lung agenesis associated with sinus-venosus defect. Other malformations were excluded by sonographic methods. Mediastinal shift with dextropositio cordis in compensatory emphysema of the left lung and frequent infections were the cardinal symptoms. These led to cardiopulmonary decompensation twice. The implantation of the tissue-expander was performed following an aortopexy, which was only temporarily successful. This method allows the filler to be adapted with age to the pleural volume by instillation of sterile saline solution via a microport. Whether or not thorax deformities and scoliosis will be prevented cannot be predicted exactly at this time.

[Endoventricular patch-plasty for reconstruction of ventricular geometry and pump function in myocardial aneurysm]. / Endoventrikuläre Patchplastik zur Wiederherstellung von Ventrikelgeometrie und Pumpfunktion beim Herzwandaneurysma.

This report deals with the first experience with endoventricular patch plasty in patients suffering from left ventricular aneurysm. The early postoperative results in 18 patients operated upon during January and May 1995 are encouraging. All patients survived the procedure. In contrast to patients operated on according to a linear resection technique the postoperative outcome and the early results are much better with this technique. We recommend the endoventricular patch plasty on a beating heart.