Humans ; Pulmonary Atresia/therapy* ; Pregnancy ; Female ; China ; Consensus ; Fetal Heart ; Ultrasonography, Prenatal ; Heart Defects, Congenital/therapy* ; Ventricular Septum ; Cardiac Surgical Procedures/methods*

Decreased exercise capacity after Fontan surgery is relatively common and the failure of the Fontan state gradually increases with age. However, there is no further treatment for patients with Fontan circulation. Pulmonary vasodilation therapy is an effective method to solve this problem because pulmonary vascular resistance is a major factor of the Fontan problem. Inhaled iloprost is a chemically stable prostacyclin analogue and a potent pulmonary vasodilator. We experienced two cases of Fontan patients treated with inhaled iloprost for 12 weeks. The first patient was an 18-year-old female with pulmonary atresia with an intact ventricular septum, and the second patient was a 22-year-old male with a double outlet right ventricle. Fifteen years have passed since both patients received Fontan surgery. While the pulmonary pressure was not decreased significantly, improved exercise capacity and cardiac output were observed without any major side effects in both patients. The iloprost inhalation therapy was well tolerated and effective for the symptomatic treatment of Fontan patients.
Adolescent ; Cardiac Output ; Double Outlet Right Ventricle ; Epoprostenol ; Female ; Fontan Procedure ; Humans ; Iloprost* ; Male ; Pulmonary Atresia ; Respiratory Therapy ; Vascular Resistance ; Vasodilation ; Ventricular Septum ; Young Adult

OBJECTIVETo assess the feasibility of transcatheter intervention for critical pulmonary arterial valve stenosis and atresia with intact ventricular septum in neonates.

METHODSFrom June 2006 to January 2008, 13 neonates (9 with critical pulmonary arterial valve stenosis, 4 with pulmonary arterial valve atresia) underwent transcatheter intervention(one of them was premature neonate). Ten of the patients were boys and 3 were girls. The mean age was (17.4 +/- 10.7) days. The mean weight was (3.4 +/- 0.8) kg. All the patients received prostaglandin E(1) infusion to dilate the ductus arteriosus before and during the procedure. Valvuloplasty was performed using a low-profile balloon (2.5 - 4.0 mm in diameter), and then a balloon approximately 1.0-1.2 times the annulus diameter was applied. In patients with pulmonary atresia, the valve was perforated with a radiofrequency wire, and then valvuloplasty was performed.

RESULTSTwelve patients (92%) were successfully treated with transcatheter intervention. Right ventricular systolic pressure decreased by 50% [from (120.1 +/- 17.0) mm Hg (1 mm Hg = 0.133 kPa) to (58.8 +/- 7.7) mm Hg, P < 0.001], and the ratio of the right ventricular systolic pressure to systemic systolic pressure decreased by 44% [from (1.6 +/- 0.2) to (0.9 +/- 0.1), P < 0.001]. The right ventricular angiography showed that the pulmonary valves were opened. The ratio of the balloons to annulus were (1.1 +/- 0.1) (the maximal balloon size range from 6 to 12 mm). The procedure lasted for a mean of (127.5 +/- 32.6) minutes. The duration of fluoroscopy was (25.2 +/- 7.2) minutes. Complications occurred in 4 patients (30%). Hemopericardium occurred in 2 patients, hypoxia in 1 patient and supraventricular tachycardia in 1 patient. One (8%) patient died. The follow-up periods were (7.2 +/- 6.4) months. Three patients had moderate to severe residual pulmonary stenosis. One of them underwent a second balloon dilation at 3 months of follow-up. The other two were waiting for the second dilation. Nine patients had mild to moderate residual pulmonary stenosis. All patients had mild pulmonary regurgitation. Ductus arteriosus was naturally closured in 11 patients, and nearly closured in 1 patient.

CONCLUSIONSTranscatheter intervention for critical pulmonary stenosis and atresia with intact ventricular septum in neonates is safe and effective. Some patients may require repeat balloon valvuloplasty.

Catheterization ; Critical Illness ; Feasibility Studies ; Female ; Humans ; Infant, Newborn ; Male ; Pulmonary Atresia ; therapy ; Pulmonary Valve Stenosis ; therapy ; Treatment Outcome

OBJECTIVETo investigate the efficacy and safety of percutaneous radiofrequency perforation and valvuloplasty in infants with pulmonary atresia with intact ventricular septum (PA/IVS).

METHODSFour infants (body weight 4 - 10 kg) aged 11 months, 9 months, 12 days and 9 months old, respectively, were hospitalized for dyspnea and cyanosis. All patients had a continuous murmur in the left second intercostal space. Doppler echocardiogram showed membranous pulmonary atresia with intact ventricular septum. Right ventriculogram showed a tripartite right ventricle, vasiform infundibulum, and membranous pulmonary valve atresia without ventriculocoronary connections. Descending thoracic aortogram showed good-sized confluent pulmonary arteries being filled from a ductus arteriosus. All the patients were taken up for radiofrequency perforation followed by a balloon dilatation. A 6F Judkins right coronary guiding catheter was positioned in the right ventricular outflow tract and under the atretic pulmonary valve membrane. The radiofrequency perforation catheter along with coaxial injectable catheter was then passed through the right coronary guiding catheter, using it as the guide to the imperforate membrane. The proximal end of the radiofrequency perforation catheter was then connected to radiofrequency generator. After the cusps of pulmonary valve were perforated, the coaxial injectable catheter was moved into the main pulmonary artery. A tiny floppy-tipped coronary guidewire was then passed through the coaxial injectable catheter into the main pulmonary artery and directed through the patent ductus arteriosus into the descending thoracic aorta or directed into pulmonary arteriola. Thereafter, serial balloon dilation catheters were introduced across the pulmonary valve, and dilations were sequentially performed with increasing balloon diameters. The balloon was dilated until the concave of the balloons disappeared. The radiofrequency energy (5 to 8 W) was delivered for 2 to 5 seconds once, but commonly twice, to perforate the valves. After a predilation with a 3 mm x 20 mm to 5 mm x 20 mm balloon at 6 - 14 atm pressure, the valve was subsequently dilated with 10 mm x 30 mm to 14 mm x 30 mm balloon once or twice. The duration of procedures was 120 to 150 min and exposure time was 25.4 to 43.9 min.

RESULTSThe primary procedure was successful in all the infants except one who died early of cardiac perforation with tamponade. After a follow-up period ranging from 2 to 8 months (mean 4.3 m), the remaining 3 survivors achieved complete biventricular circulation. Two of them were awaiting occlusion of the patent ductus arteriosus and 1 needed right ventricular outflow tract reconstruction because of infundibular obstruction.

CONCLUSIONPA/IVS consists of 0.7% to 3.1% of congenital heart defects. 85% of the untreated patients die within half a year. Surgical repair for the infants with PA/IVS is associated with a high mortality. In carefully selected patients with PA/IVS, radiofrequency perforation and balloon dilatation of the pulmonary valve is feasible and may represent a new alternative to surgery due to its low mortality and avoidance of cardiopulmonary bypass.

Balloon Occlusion ; Catheter Ablation ; methods ; Catheterization ; methods ; Female ; Humans ; Infant ; Infant, Newborn ; Male ; Pulmonary Atresia ; physiopathology ; therapy ; Pulmonary Valve ; surgery ; Ventricular Septum

OBJECTIVEThe advances in interventional cardiac catheterization have changed the therapeutic strategy for many patients with congenital heart diseases. The aim of this study was to evaluate the role of therapeutic cardiac catheterization in the treatment of complex congenital heart diseases.

METHODSBalloon atrial septostomy (BAS) was performed in 59 children using Rashkind balloon catheter. Static balloon dilatation of the atrial septum was performed in 2 children with hypoplastic right heart syndrome. One child with pulmonary artery stenosis at the suture lines after arterial switch was treated with balloon dilatation. Percutaneous balloon pulmonary valvuloplasty (PBPV) and balloon angioplasty were performed in 15 children with tetralogy of Fallot (TOF). Transcatheter coil embolization was performed in 18 children with systemic to pulmonary collateral vessels and 5 children with B-T shunts before surgical procedures. Transcatheter closure of fenestration with Amplatzer septal occluder device was performed in 1 child who had undergone Fontan procedure.

RESULTSIn 46 children with transposition of great arteries (TGA), the arterial oxygen saturation (SaO(2)) was increased from 0.57 +/- 0.17 to 0.76 +/- 0.13 (t = 14.58, P < 0.01) after BAS. The pressure gradients across left and right atrium were less than 2 mmHg. The created atrial septal defects were 5 - 20 mm in size. In 10 children with pulmonary atresia with intact ventricular septum (PA/IVS), the arterial oxygen saturation did not change after BAS (t = 1.57, P > 0.05), but the pressure gradients across left and right atrium were less than 2 mmHg. In children with TOF, the arterial oxygen saturation was increased by 15 percent after PBPV and pulmonary valvular stenosis was relieved. In 14 of 18 children with systemic to pulmonary collateral vessels and 5 children with B-T shunts, complete occlusion was accomplished and the procedures were successful. In the child who had undergone Fontan procedure, the fenestration was occluded successfully and no complication was observed.

CONCLUSIONIn management of complex congenital heart diseases, combination of surgical procedure and interventional catheterization therapy could be suggested to have better outcome.

Cardiac Catheterization ; methods ; Catheterization ; Child ; Heart Defects, Congenital ; therapy ; Humans ; Prosthesis Implantation ; Pulmonary Atresia ; therapy ; Pulmonary Valve Stenosis ; therapy ; Tetralogy of Fallot ; therapy ; Transposition of Great Vessels ; therapy