Background— Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare congenital lesion with high mortality. Therapy was exclusively surgical until recently, when the use of radiofrequency-assisted perforation of the atretic valve was introduced as a treatment option. This study analyzes the outcomes and morphological changes to right heart structures after percutaneous perforation and balloon dilation of the atretic valve. Methods and Results— Between April 1992 and August 2000, 30 patients with PA-IVS underwent attempted percutaneous valve perforation and balloon dilation of the pulmonary valve. Longitudinal echocardiographic measurements of the tricuspid valve diameter, right ventricular length and area were recorded. Z scores were calculated according to published formulas. Perforation was achieved in 27 patients. In 14 patients a modified Blalock-Taussig shunt was performed between 2 and 24 days after valve dilation. There were 3 early and 2 late deaths. Among the survivors (follow-up time of 1 to 87 months), 16 patients had a biventricular circulation, 3 a 1 ½-ventricle circulation, and 1 a Fontan operation. Four patients are awaiting further palliation. There was no significant change of the tricuspid valve Z score or right ventricular length Z score with time. Conclusions— Percutaneous balloon valvotomy is an effective treatment strategy for patients with PA-IVS provided that there is a patent infundibulum and a lack of a right ventricle–dependent coronary circulation. Despite the observation that right heart growth does not increase with body growth in early follow-up, it appears adequate to maintain a biventricular circulation in many patients.
This is the first report on histopathology of a septal defect occluder with a bioresorbable filament structure in vivo which is already in clinical use. Good biocompatibility was demonstrated with documentation of timely degradation and substitution of the polymer material by fibromuscular cells and extracellular matrix components.
In a retrospective study of 51 consecutive patients undergoing transcatheter closure of secundum type atrial septal defects (ASDs), we examined the reliability of transesophageal echocardiography (TEE) prior to catheterization and compared the diameter with that obtained by balloon measurement during catheterization. The TEE diameter was 16.3 ± 4.6 mm compared with 22.5 ± 6.0 mm for the stretched diameter obtained during catheterization (p < 0.001). There was no gender difference. The degree of left-to-right shunting correlated poorly with the size of the defect. We conclude that although TEE is accurate for diagnosis of an ASD, the measurement of its size to determine the size of the closure device is at best inaccurate.
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