Importance of right ventricular outflow tract angiography in distinguishing critical pulmonary stenosis from pulmonary atresia.

Abstract

Objective-To investigate the spectrum of pulmonary atresia and critical pulmonary stenosis using right ventricular outflow tract angiography and explore its implications for catheter interventional treatment.Design-Prospective clinical study. Setting Two paediatric cardiology centres.Subjects-11 neonates or infants (aged 1 day to 8 months; weighing 2 3 to 7'8 kg) with pulmonary atresia or where the differentiation of pulmonary atresia from critical pulmonary stenosis was unclear on either echocardiography or angiography. Methods-Right ventricular outflow tract angiography was performed on all patients to distinguish pulmonary atresia from critical pulmonary stenosis before opening the right ventricular outflow tract.Results-Right ventricular outflow tract angiography showed that three of seven patients diagnosed as pulmonary atresia by echocardiography had pin hole jets across the pulmonary valve; another had a probe patent valve that appeared imperforate on both echocardiography and right ventricular outflow tract angiography. Three of the four patients diagnosed by echocardiography as critical pulmonary stenosis were found on right ventricular outflow tract angiography to have pulmonary atresia. The remaining patient had such a tiny orifice that a second orifice had to be created with a radiofrequency catheter. The right ventricular outflow tract was opened successfully in 10 of the 11 patients, six of whom required application of radiofrequency energy. The right ventricular to aortic systolic pressure ratio fell from 1-4 (0.9 to 1-9) to 0-6 (0.2 to 1.1) (P < 005). All 11 patients were alive and well with transcutaneous oxygen saturations ranging from 84% to 95% at a median follow up duration of nine months.Conclusions-Critical pulmonary stenosis and pulmonary atresia cannot always be accurately distinguished by echocardiography. Right ventricular outflow tract angiography helps to distinguish the two groups. In most cases the right ventricular outflow tract can be opened without mortality and with short to medium term survival. (Heart 1997;77:456-460) Keywords: pulmonary atresia; critical pulmonary stenosis; radiofrequency perforation; angiography Pulmonary atresia with intact ventricular septum remains a difficult condition to treat. Prognosis and management depends on the degree of ventricular hypoplasia and number of parts of the ventricular cavity remaining, and in particular whether the infundibulum is present.' 2 Right ventricular to coronary artery sinusoids with coronary artery stenoses may produce a right ventricular dependent myocardial blood supply, preventing right ventricular decompression.3 Surgical strategies consist of systemic to pulmonary shunt, with or without pulmonary valvotomy or right ventricular outflow patch, depending on the presence of an infundibulum.4 During closed surgical pulmonary valvotomy surgeons often remark that there is a tiny jet of blood coming through a pin hole orifice. The distinction between critical pulmonary stenosis and pulmonary atresia may not be com...

Pulmonary Atresia with Intact Ventricular Septum: Management Options and Decision-making

“…Pulmonary angiogram using a catheter across PDA will delineate main and branch pulmonary arteries. Aortopulmonary collateral arteries are rare in PA-IVS [8].…”

Radiofrequency perforation in the treatment of congenital heart disease

“…In the absence of a right ventricular–dependent coronary circulation, decompression of the right ventricle is a component of a treatment algorithm, which attempts to salvage the right heart as a component of a biventricular or so‐called one‐and‐a‐half ventricular repair [36]. An alternative to primary surgical decompression strategies is the use of percutaneous laser or RF‐assisted perforation of the atretic valve and subsequent balloon dilation [5–9, 17, 19– 26, 28, 29, 37–53].…”