Percutaneous Balloon Valvotomy in Pulmonary Atresia With Intact Ventricular Septum

Abstract

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.

Restrictive Right Ventricular Physiology and Right Ventricular Fibrosis as Assessed by Cardiac Magnetic Resonance and Exercise Capacity After Biventricular Repair of Pulmonary Atresia and Intact Ventricular Septum

“…Endocardial and myocardial abnormalities of the right ventricle are well documented in PAIVS, which include massive hypertrophy, 11 extensive myocardial fiber disarray, 12 and endocardial fibroelastosis. While growth in RV size has been shown to occur after RV decompression, 6,7 complete normalization of the histologic abnormalities seems unlikely. Late gadolinium enhancement has been regarded as a marker of myocardial fibrosis and scarring.…”

Indications for Cardiac Catheterization and Intervention in Pediatric Cardiac Disease

“…A vezetődrót merev végét, transseptalis tűket, lézert vagy -a közelmúltban született beszámolók szerint -rádiófrekvenciás perforációs katétereket vagy dróto-kat használva át lehet jutni az atreticus pulmonalis billentyűn. [96][97][98][99] A beavatkozás nagy odafigyelést igényel, és bizonyos intézményekben a hibrid megközelítést alkalmazó beavatkozás részeként végzik el.…”

Cateterismo intervencionista na estenose valvar pulmonar crítica do recém-nascido e na atresia pulmonar com septo interventricular íntegro: 13 anos de experiência de um serviço terciário

“…Na atresia pulmonar com septo interventricular íntegro, os avanços tiveram início crescimento ventricular e do anel valvar pulmonar 3 , além do ganho, segundo alguns, do z score da valva tricúspide. 10 As taxas de sucesso são cada vez maiores com menores taxas de reintervenção, principalmente com o advento de novas estratégias de valvotomia com radiofrequência. Isso se deve também ao melhor entendimento da fisiologia ventricular e à seleção de casos favoráveis à perfuração da valva pulmonar.…”

“…A taxa de mortalidade hospitalar dos pacientes de 10,6% (5/47) é similar à descrita na literatura 10,14,16,17 e evidencia a gravidade da doença mesmo com adequada seleção de pacientes. Em se tratando apenas de casos de atresia pulmonar com septo interventricular íntegro, a mortalidade sobe para 23,5% (4/17).…”