Humans ; Pulmonary Valve Stenosis/therapy* ; Infant, Newborn ; Perioperative Care/standards* ; Consensus ; Balloon Valvuloplasty/methods* ; Pulmonary Valve/surgery*

No abstract available.
Anti-Bacterial Agents/therapeutic use ; Biopsy ; Echocardiography, Doppler, Color ; Echocardiography, Transesophageal ; Endocarditis, Bacterial/complications/diagnosis/drug therapy/*microbiology ; Fluorescent Antibody Technique ; Heart Septal Defects, Ventricular/*complications/diagnosis/surgery ; Humans ; Male ; Middle Aged ; Predictive Value of Tests ; Pulmonary Valve Stenosis/*complications/diagnosis ; Purpura, Schoenlein-Henoch/diagnosis/drug therapy/*etiology ; Risk Factors

OBJECTIVETo investigate the clinical characteristics, diagnosis and therapy of Keutel syndrome, and thereby to minimize the misdiagnosis.

METHODData of a case of Keutel syndrome diagnosed at the Provincial Hospital Affiliated to Shandong University were analyzed and related literature were reviewed.

RESULTAn 8-month-26-day-old boy was presented with inspiratory and expiratory stridor and wheezing after movement on lung auscultation. His craniofacial appearance was characterized by midfacial hypoplasia with a broad depressed nasal bridge. The nose was small and flat. A grade 2-3/6 systolic murmur was heard between the second and third ribs at left edge of the sternum. The end phalanges of his fingers were thickened. Chest radiograph showed tracheobronchial cartilage calcification and tracheobronchial stenosis. Echocardiographic examination revealed the right pulmonary stenosis. With endoscopic surgery, antiobstructive and antibiotic therapy clinical symptoms were improved. Three weeks later he died of lung reinfection after he was discharged from our hospital. English literature search with "Keutel syndrome" as the key word at "PubMed" showed 22 articles covering 26 patients, and the clinical symptoms were hearing loss (91%), persistent respiratory symptoms (68%), recurrent otitis media/sinusitis (67%), growth development delay (52%) in turn, and signs were brachytelephalangism (100%), low nasal bridge (95%), midfacial hypoplasia (93%), cardiac murmur (69%), and auxiliary examinations showed abnormal cartilage calcification (100%), pulmonary arterial stenosis (72%), tracheobronchial stenosis (50%).

CONCLUSIONThe diagnosis of Keutel syndrome should be considered in patients with brachytelephalangism, abnormal cartilage calcification, peripheral pulmonary stenosis, and midfacial hypoplasia. Tracheal stenosis was main clinical manifestation in part of patients.

Abnormalities, Multiple ; diagnosis ; diagnostic imaging ; therapy ; Bone and Bones ; diagnostic imaging ; Calcinosis ; diagnosis ; diagnostic imaging ; therapy ; Cartilage ; diagnostic imaging ; Cartilage Diseases ; diagnosis ; diagnostic imaging ; therapy ; Diagnosis, Differential ; Hand Deformities, Congenital ; diagnosis ; diagnostic imaging ; therapy ; Humans ; Infant ; Male ; Pulmonary Valve Stenosis ; diagnosis ; diagnostic imaging ; therapy ; Radiography, Thoracic ; Retrospective Studies ; Tomography, X-Ray Computed ; Tracheal Stenosis ; diagnosis ; diagnostic imaging

OBJECTIVETo evaluate the effect and results of short and medium periods of follow-up of percutaneous balloon pulmonary valvuloplasty for critical pulmonary stenosis of neonates and infants under 6 months of age.

METHODSBetween January 2002 and December 2008, 34 consecutive patients aged from 13 to 175 days with critical pulmonary valvular stenosis underwent percutaneous balloon valvuloplasty. Patients records, catheterization data, angiograms and echocardiograms were reviewed. Patients were followed up for 6 months to 4 years (mean 25.5 months) by means of clinical examination and Doppler echocardiography.

RESULTSThe pulmonary valvuloplasty was accomplished in 32 (94%) of 34 attempts. Immediately after dilation, right ventricular systolic pressure (RVSP) decreased from (96 ± 28) mm Hg (1 mm Hg = 0.133 kPa) (49 ± 20) mm Hg (P < 0.01), the transvalvular peak to peak systolic gradient (ΔP) decreased from (89 ± 25) mm Hg to (25 ± 12) mm Hg (P < 0.01), and the right ventricular/aortic systolic pressure ratio decreased from 1.2 ± 0.5 to 0.7 ± 0.3 (P < 0.01). One patient died because of cardiac tamponade following rupture of the pulmonary valve annulus, 2 patients developed pericardial effusion, 3 patients had infundibular spasm, 3 patients had a pre-dilation by small balloon and 1 patient had weakened femoral artery pollex. After a follow up period of 6 months to 4 years 3 of 31 patients lost to follow-up. Repeat valvuloplasty was performed in 5 patients (3 neonates), no patient required surgery, and the other 23 patients did not undergo further intervention, a mean peak systolic Doppler gradient of (20 ± 13) mm Hg was found and no significant pulmonary regurgitation was seen.

CONCLUSIONSPercutaneous balloon pulmonary valvuloplasty was effective and safe for the treatment of critical pulmonary stenosis of neonates and infants under 6 months of age with good short and medium term results.

Catheterization ; Female ; Follow-Up Studies ; Humans ; Infant ; Infant, Newborn ; Lost to Follow-Up ; Male ; Pulmonary Valve ; Pulmonary Valve Stenosis ; therapy ; Treatment Outcome

OBJECTIVEAlthough NuMED Cheatham-Platinum (CP) stent was specifically designed to treat vascular obstructions associated with congenital heart disease (CHD), its application in pediatric patients is relatively uncommon, especially in the pulmonary artery stenosis. The aim of this study was to evaluate the immediate-, early- and intermediate-term results of CP stent implantation in the treatment of vessel stenosis associated with CHD in children and adolescents.

METHODSFrom August 2005 to May 2007, 5 consecutive patients (3 boys and 2 girls) diagnosed as vascular stenosis associated with CHD underwent CP stent implantation in our institution. One patient had native coarctation of the aorta (CoA) and four patients had pulmonary artery stenosis. The median age and weight of patients were 12 years (range 4 - 15 years) and 24 kg (range 20 - 51 kg), respectively. The CP stent and NuMED Balloon-in-Balloon catheter were selected according to digital subtracted angiography measurements. After checking for correct position by angiography, the inner balloon and outer balloon inflated successively to expand the stent to desired diameter.

RESULTSTotally 6 stent placement procedures were performed and 8 CP stents (8-zig, 22 - 39 mm in length) were implanted in these 5 patients. All stents but one in a case of right pulmonary artery stenosis were immediately successfully placed in the target lesions without displacement during the procedures. For this case, a repeat procedure was performed and a second CP stent was reimplanted successfully 11 months later. After the procedure, the systolic pressure gradient across the stenosis decreased from (43.43 +/- 25.61) mm Hg (1 mm Hg = 0.133 kPa) to (3.29 +/- 3.09) mm Hg (t = 4.320, P < 0.01) and the narrowest diameter of the stenotic vessels increased from (6.86 +/- 2.04) mm to (13.44 +/- 4.02) mm (t = -4.508, P < 0.01). The percentage of pulmonary artery flow to the ipsilateral lung increased from 11.0% and 13.0% to 47.5% and 52.2% after the procedure in 2 cases of unilateral pulmonary artery branch stenosis, respectively. The ratio of right ventricular to aortic systolic pressure decreased from 62.3% and 72.2% to 27.0% and 33.3% in 2 cases of bilateral branch pulmonary artery stenosis, respectively. Upper limb blood pressure of one case of native CoA dropped greatly from 206/133 mm Hg to 156/95 mm Hg. During a median follow-up of 20 months (range 13 - 34 months), the results have been stable without complications except 2 stents which developed intrastent restenosis 6 months after the procedure.

CONCLUSIONOur experience suggests that the CP stent implantation is safe and feasible for the treatment of vessel stenosis associated with CHD in children and adolescents. The immediate-, early- and interim results are encouraging, but long-term results remain to be further evaluated and demand many more cases to be studied.

Adolescent ; Aortic Coarctation ; therapy ; Cardiac Catheterization ; Child ; Child, Preschool ; Constriction, Pathologic ; Female ; Heart Defects, Congenital ; complications ; therapy ; Humans ; Male ; Platinum ; Pulmonary Valve Stenosis ; complications ; therapy ; Stents ; Treatment Outcome

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

PURPOSE: We report herein major determinants and long- term outcomes of balloon dilatation (BD) for 27 pediatric patients with isolated native valvular pulmonary stenosis (VPS). MATERIALS AND METHODS: From May 1997 to May 2003, 27 pediatric patients with VPS (pressure gradients> or =40mmHg) were enrolled in this retrospective study. Single-balloon maneuver was applied in 26 patients, and double- balloon maneuver in 1. After BD, the pressure gradients were documented simultaneously by pullback maneuver by cardiac catheterization and echocardiography within 24 hours, at 1- month, 3-month, 1-year, and 4-to-10-year follow-ups. RESULTS: Before BD, the echocardiographic gradients ranged from 40 to 101mmHg (61+/-19, 55), and from 40 to 144mmHg (69+/-32, 60) by pressure recordings. After BD, the gradients ranged from 12 to 70mmHg (29+/-13, 27) by pressure recording (p<0.001), and from 11 to 64mmHg (27+/-12, 26) by echocardiography within 24 hrs (p<0.001). The ratios of the systolic pressure of the right ventricle to those of the left ventricle were 55 to 157% (89+/-28, 79%) before BD, and 30 to 79% (47+/-13, 42%) after BD p<0.001). Follow-up (7.7+/-5.7, 4.5 years) echocardiographic gradients ranged from 11 to 61mmHg (25+/-11, 24). Two patients did not have immediate success owing to infundibular spasm. Improved right ventricular compliance could be accounted for the ultimate success in these 2 patients. The ultimate successful rate was 100%. CONCLUSION: BD can achieve excellent long-term outcomes in the pediatric patients with isolated native VPS.
Adolescent ; Balloon Dilatation/adverse effects/*methods ; Child ; Child, Preschool ; Echocardiography ; Female ; Follow-Up Studies ; Humans ; Infant ; Infant, Newborn ; Male ; Pulmonary Valve Stenosis/pathology/physiopathology/*therapy ; Retrospective Studies ; Time Factors ; Treatment Outcome

OBJECTIVETo assess exercise capacity after percutaneous balloon pulmonary valvuloplasty (PBPV) in children with isolated pulmonary valve stenosis.

METHODSTreadmill exercise testing was performed in 46 children (6.3 +/- 3.1 years, ranging 1 to 12.5 years) with isolated pulmonary valve stenosis post PBPV and in 294 healthy children. Exercise capacity, change in heart rate, blood pressure, and electrocardiogram during exercise were observed.

RESULTSPost PBPV, the transvalvular gradient was below 25 mm Hg (1 mm Hg = 0.133 kPa) in all patients. The mean work capacity in post PBPV children was about 90 percent of that in healthy children. Heart rate and blood pressure response to exercise is comparable to healthy children. Factors associated with exercise capacity include age at operation and the ratio of balloon/pulmonary valve diameter. Exercise-induced uniform ventricular premature contraction in 5 children post PBPV and no ST segment change was observed.

CONCLUSIONExercise capacity post percutaneous balloon pulmonary valvuloplasty in children with isolated pulmonary valve stenosis was comparable to that in healthy children.

Adolescent ; Angioplasty, Balloon ; Blood Pressure ; Child ; Child, Preschool ; Electrocardiography ; Exercise Tolerance ; Female ; Follow-Up Studies ; Heart Rate ; Humans ; Infant ; Male ; Pulmonary Valve Stenosis ; physiopathology ; therapy

Femoral artery thrombosis is an uncommon but potentially serious complication following pediatric cardiac catheterization. Treatment options of thrombosis include intravenous heparin infusion, thrombolytic therapy such as streptokinase, urokinase and tissue plasminogen activator, and surgical thrombectomy. These treatments have varying degrees of success and complication. We report successful use of recombinant tissue plasminogen activator for management of femoral artery thrombosis in neonate following transcatheter balloon valvuloplasty of pulmonary valvular stenosis.
Balloon Valvuloplasty ; Cardiac Catheterization ; Cardiac Catheters ; Constriction, Pathologic ; Femoral Artery* ; Heparin ; Humans ; Infant, Newborn* ; Pulmonary Valve Stenosis ; Streptokinase ; Thrombectomy ; Thrombolytic Therapy ; Thrombosis* ; Tissue Plasminogen Activator* ; Urokinase-Type Plasminogen Activator

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