Stenting of the left pulmonary venous baffle obstruction was successfully performed in a 6 year old girl after the Mustard operation for transposition of the great arteries. She also had stent implantation in both the superior vena cava and inferior vena cava baffle junctions. These procedures were followed by sustained clinical improvement. Angiography showed that all three stents were fully patent a year after the procedure. (Heart 1996;75:210-212
Balloon dilatation was successfully performed in two patients with complete obstruction of the superior vena cava baffle junction after a Mustard operation for transposition of the great arteries. Evidence for complete relief of obstruction in the first patient, aged 4 years, was obtained by angiography, which showed improved calibre at the site of obstruction and improved haemodynamic pressure measurement after the balloon dilatation. In the second patient, aged 14 years, the relief was incomplete; in this patient a 3 cm long 3 mm diameter Palmaz stent was successfully implanted. (Br Heart _' 1994;72:482-485) For many years the Mustard operation was the preferred operation for physiological repair of transposition of the great arteries. Complications of this operation include obstruction of both systemic and pulmonary venous pathways.' 2 Reoperation is recommended for obstructions of the pulmonary vein inferior vena cava, and superior vena cava if symptomatic.3 Successful balloon dilatation of incomplete systemic venous obstruction has been reported.245Balloon dilatation was successfully performed in two patients with complete obstruction of the superior vena cava after the Mustard procedure for transposition of the great arteries. The obstructed end was pierced with a super stiff guidewire in case 1, and a transseptal needle in case 2.
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...
A child diagnosed with transposition of great arteries, multiple (Swiss cheese) ventricular septal defects, and a small right ventricle underwent pulmonary artery banding and patent ductus arteriosus ligation at the age of six months. At the age of three years bidirectional cavopulmonary shunt was performed as a first stage for univentricular repair. However, the patient was lost follow-up for four years, following which further evaluation showed that the right ventricle was reasonably adequate to support pulmonary circulation if the ventricular septal defects (VSDs) were closed using percutaneous techniques. Four VSDs were then closed using Amplatzer devices (AGA Medical Corporation, Plymouth, MN, USA). At the age of eight years she underwent complex biventricular repair in the form of arterial switch, closure of atrial septal defect, take down of Glenn shunt, and reanastomosis of the distal end of the superior vena cava to the distal superior vena cava stump on the right atrium. One year later the patient is alive and well. In conclusion; biventricular repair may be considered before completion of Fontan whenever cardiac anatomy allows.
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