Objective: To evaluate the effectiveness and safety of Pul-Stent as the treatment of postoperative branch pulmonary artery stenosis in children with congenital heart disease. Methods: This was a retrospective study. Thirty-three patients who underwent Pul-Stent implantation in Shanghai Children's Medical Center due to postoperative residual pulmonary artery stenosis from August 2014 to June 2015 were included. The immediate curative effect, follow-up and complications of Pul-Stent implantation were assessed. Comparisons between groups were performed with unpaired Student t test. Results: Pul-Stent implantation of 33 patients (19 males and 14 females) were performed successfully. Thirty-one patients underwent percutaneous stenting, and 2 patients underwent hybrid stenting. A total of 35 Pul-Stents were implanted (19 of model small, 15 of model medium and one of model large), 23 stents were planted in the proximal left pulmonary artery and 12 stents were in the proximal right pulmonary artery. The initial diameter of dilation balloon ranged from 6 to 16 mm, and the long sheath of percutaneous implantation ranged from 8 to 10 F in 29 patients (29/31, 94%). After stenting, the diameter of the narrowest segment of pulmonary artery increased from (4.0±1.7) mm to (9.1±2.1) mm in all patients (t=-21.60, P<0.001). The pressure gradient at the stenosis in 26 patients after biventricular correction decreased from (30.5±12.3) mmHg (1 mmHg=0.133 kPa) to (9.9±9.6) mmHg (t=12.92, P<0.001), and the right ventricular to aortic pressure ratio decreased from 0.57±0.14 to 0.44±0.12 (t=7.44, P<0.001). The pressure of the superior vena cava after stenting in 5 patients after cavopulmonary anastomosis decreased from (17.0±1.9) mmHg to (14.0±0.7) mmHg (t=2.86, P=0.046). Two patients died during reoperation for repairing other cardiac malformations. The remaining 31 patients were clinically stable during the follow-up period of (5.3±1.6) years, and one stent fracture was found on chest X-ray. Cardiac catheterization reexaminations in 16 patients showed that restenosis was found in one stent, while stent position and patency were satisfactory in the remaining stents. Nine children underwent post-dilation without stent fracture, displacement or aneurysm formation. Cardiac tomography showed no stent stenosis, fracture observed, or significant change in diameter of the stent in 8 patients. The inner diameter and pulmonary blood perfusion could not be accurately evaluated due to artifacts by cardiac magnetic resonance imaging in 4 patients. Conclusions: Pul-Stent has good compliance and adequate radial strength, and can dilate further over time to accommodate for somatic growth. It performs safely and effectively in treating post-operative branch pulmonary artery stenosis in children.
Child ; China ; Female ; Follow-Up Studies ; Heart Defects, Congenital/surgery* ; Humans ; Male ; Pulmonary Artery/surgery* ; Retrospective Studies ; Stenosis, Pulmonary Artery/surgery* ; Stents ; Treatment Outcome ; Vena Cava, Superior

PURPOSE: The present study aimed to evaluate progression and prognosis according to the palliation method used in neonates and early infants aged 3 months or younger who were diagnosed with pulmonary atresia with ventricular septal defect (PA VSD) or tetralogy of Fallot (TOF) with severe pulmonary stenosis (PS) in a single tertiary hospital over a period of 12 years. METHODS: Twenty with PA VSD and 9 with TOF and severe PS needed initial palliation. Reintervention after initial palliation, complete repair, and progress were reviewed retrospectively. RESULTS: Among 29 patients, 14 patients underwent right ventricle to pulmonary artery (RV-PA) connection, 11 palliative BT shunt, 2 central shunt, and 2 ductal stent insertion. Median age at the initial palliation was 13 days (1–98 days). Additional procedure for pulmonary blood flow was required in 5 patients; 4 additional BT shunt operations and 1 RV-PA connection. There were 2 early deaths among patients with RV-PA connection, one from RV failure and the other from severe infection. Finally, 25 patients (86%) had a complete repair. Median age of total correction was 12 months (range, 2–31 months). At last follow-up, 2 patients had required reintervention after total correction; 1 conduit replacement and 1 right ventricular outflow tract (RVOT) patch enlargements. CONCLUSION: For initial palliation of patients with PA VSD or TOF with severe PS, not only shunt operation but also RV-PA connection approach can provide an acceptable outcome. To select the most proper surgical strategy, we recommend thorough evaluation of cardiac anomalies such as RVOT and PA morphologies and consideration of the patient's condition.
Follow-Up Studies ; Heart Septal Defects, Ventricular* ; Heart Ventricles ; Humans ; Infant ; Infant, Newborn ; Methods ; Palliative Care ; Prognosis ; Pulmonary Artery ; Pulmonary Atresia* ; Pulmonary Valve Stenosis* ; Retrospective Studies ; Stents ; Tertiary Care Centers ; Tetralogy of Fallot*

BACKGROUND AND OBJECTIVES: We investigated the effectiveness of balloon dilatation of homograft conduits in the pulmonary position in delaying surgical replacement. SUBJECTS AND METHODS: We reviewed the medical records of patients who underwent balloon dilatation of their homograft in the pulmonary position from 2001 to 2015. The pressure gradient and ratio of right ventricular pressure were measured before and after the procedure. The primary goal of this study was to evaluate the parameters associated with the interval to next surgical or catheter intervention. RESULTS: Twenty-eight balloon dilations were performed in 26 patients. The median ages of patients with homograft insertion and balloon dilatation were 20.3 months and 4.5 years, respectively. The origins of the homografts were the aorta (53.6%), pulmonary artery (32.1%), and femoral vein (14.3%). The median interval after conduit implantation was 26.7 months. The mean ratio of balloon to graft size was 0.87. The pressure gradient through the homograft and the ratio of right ventricle to aorta pressure were significantly improved after balloon dilatation (p<0.001). There were no adverse events during the procedure with the exception of one case of balloon rupture. The median interval to next intervention was 12.9 months. The median interval of freedom from re-intervention was 16.6 months. Cox proportional hazards analysis revealed that the interval of freedom from re-intervention differed only according to origin of the homograft (p=0.032), with the pulmonary artery having the longest interval of freedom from re-intervention (p=0.043). CONCLUSION: Balloon dilatation of homografts in the pulmonary position can be safely performed, and homografts of the pulmonary artery are associated with a longer interval to re-intervention.
Allografts* ; Angioplasty, Balloon ; Aorta ; Catheters ; Dilatation* ; Femoral Vein ; Freedom ; Heart Ventricles ; Humans ; Medical Records ; Pulmonary Artery ; Pulmonary Valve Stenosis ; Rupture ; Transplants ; Ventricular Pressure

Objective To assess the application of high-pitch CT pulmonary angiography (CTPA) at 70 kV tube voltage with 15 ml contrast medium using third-generation dual-source CT. Methods A total of 70 patients with clinically suspected pulmonary embolism were randomly divided into two groups: group A (n=35) underwent CTPA on conventional scanning mode (120 kV,80 ml contrast medium);and group B (n=35) underwent CTPA on high-pitch scanning mode at 70 kV tube voltage with 15 ml contrast medium. The CT values and standard deviations of the main pulmonary artery,apical segment of right upper pulmonary lobe (S1),and posterior basal segment of the right lower pulmonary lobe (S10),anterior thoracic air,and back muscles were measured. The signal to noise ratio (SNR),contrast to noise ratio (CNR),and effective dose (ED) were calculated. The overall image quality was evaluated by two blinded radiologists. The quality image was compared using non-parametric test on two independent samples. The potential differences in CT value,SNR,CNR,and ED were analyzed using the independent sample t-test. Results The CT values of main pulmonary artery [(300.62±77.54)HU vs.(332.80±102.80)HU;t=-1.53,P=0.13],S1 [(361.72±84.92)HU vs. (325.37±87.86)HU;t=1.81,P=0.08],and S10 [(359.54±89.61)HU vs. (318.26±87.19)HU;t=2.00,P=0.05] of right lung were not significantly different between group A and group B. The CNR of S1 (22.81±6.05 vs. 19.80±6.60;t=2.05,P=0.04) and S10 (22.65±6.37 vs. 19.28±6.63;t=2.23,P=0.03) of right lung in group A was significantly higher than in group B. The SNR of main pulmonary artery,S1,and S10 of right lung were not significantly different between group A and B. The subjective diagnostic quality values of group A and B were 1 (1,1) and 1 (1,1),respectively (Z=-0.08,P=0.93). The subjective diagnostic quality values evaluated by two radiologists showed excellent consistency(κ=0.87,P=0.01). The mean ED was 79% lower in group B [(0.92±0.23)mSv] than in group A [(4.33±1.80) mSv] (t=11.72,P=0.00).Conclusion Application of high-pitch mode in CTPA at 70 kV with 15 ml contrast medium using third-generation dual-source CT can remarkably reduce radiation dose without affecting image quality.
Computed Tomography Angiography ; methods ; Humans ; Lung ; diagnostic imaging ; Pulmonary Embolism ; diagnostic imaging ; Radiation Dosage ; Signal-To-Noise Ratio ; Stenosis, Pulmonary Artery ; diagnostic imaging

Williams syndrome (WS) is a developmental disorder characterized by vascular abnormalities such as thickening of the vascular media layer in medium- and large-sized arteries. Supravalvular aortic stenosis (SVAS) and peripheral pulmonary artery stenosis (PPAS) are common vascular abnormalities in WS. The natural course of SVAS and PPAS is variable, and the timing of surgery or intervention is determined according to the progression of vascular stenosis. In our patient, SVAS and PPAS showed rapid concurrent progression within two weeks after birth. We report the early manifestation of SVAS and PPAS in the neonatal period and describe the surgical treatment for stenosis relief.
Aorta ; Aortic Stenosis, Supravalvular ; Arteries ; Constriction, Pathologic* ; Heart Defects, Congenital ; Humans ; Parturition ; Pulmonary Artery* ; Tunica Media ; Williams Syndrome*

The half-turned truncal switch (HTTS) operation has been reported as an alternative to the Rastelli or réparation à l'étage ventriculaire procedures. HTTS prevents left ventricular outflow tract (LVOT) obstruction in patients with complete transposition of the great arteries (TGA) with a ventricular septal defect (VSD) and pulmonary stenosis (PS), or in those with a Taussig-Bing anomaly with PS. The advantages of the HTTS procedure are avoidance of late LVOT or right ventricular outflow tract (RVOT) obstruction, and of overstretching of the pulmonary artery. We report the case of a patient who underwent HTTS for TGA with VSD and PS, in whom there was no LVOT obstruction and only mild aortic regurgitation and mild RVOT obstruction, including observations at 12-year follow-up. Our experience with long-term follow-up of HTTS supports a solution for late complications after the Rastelli procedure.
Aortic Valve Insufficiency ; Arteries* ; Double Outlet Right Ventricle ; Follow-Up Studies* ; Heart Septal Defects, Ventricular* ; Humans ; Pulmonary Artery ; Pulmonary Valve Stenosis* ; Transposition of Great Vessels

Aged ; Balloon Valvuloplasty ; methods ; Coronary Artery Bypass, Off-Pump ; methods ; Coronary Artery Disease ; surgery ; Humans ; Male ; Middle Aged ; Pulmonary Valve Stenosis ; surgery

A five-month-old boy who had undergone previously transcatheter balloon atrioseptostomy at 3 days of age for complete transposition of the great arteries with ventricular septal defect and pulmonary stenosis underwent pulmonary root translocation with the Lecompte maneuver. This operation has the advantages of maintaining pulmonary valve function, preserving the capacity for growth, and avoiding problems inherent to the right ventricular to pulmonary artery conduit. This patient progressed well for 9 months postoperatively and we report this case of pulmonary root translocation with the Lecompte maneuver.
Arteries* ; Heart Defects, Congenital ; Heart Septal Defects, Ventricular* ; Humans ; Male ; Pulmonary Artery ; Pulmonary Valve ; Pulmonary Valve Stenosis* ; Transposition of Great Vessels

Noonan syndrome is an autosomal dominant disorder characterized by dysmorphic facial features, congenital heart defects and short stature. To date, renal artery stenosis has not been associated with Noonan syndrome. We report the case of a 27-year old male who presented with malignant hypertension associated with renal artery stenosis, dysmorphic facial features, pectus excavatum, pulmonary stenosis and hypertrophic cardiomyopathy who was diagnosed with Noonan syndrome.
Adult* ; Balloon Valvuloplasty ; Cardiomyopathy, Hypertrophic ; Funnel Chest ; Heart Defects, Congenital ; Humans ; Hypertension, Malignant* ; Male ; Noonan Syndrome* ; Pulmonary Valve Stenosis ; Renal Artery Obstruction

OBJECTIVETo discuss the diagnosis and surgical treatment of congenital vascular ring and prognostic factors.

METHODSThe clinic data of 42 cases of congenital vascular from January 2010 to December 2013 was analyzed retrospectively (accounting for 1.04% congenital heart operations over the same period ). There were 26 male and 16 female patients, aged at surgery 24 days to 6 years (average 10.7 months). The diagnosis including pulmonary artery sling in 26 cases, double aortic arch in 10 cases, right aortic arch with aberrant left subclavian artery in 3 cases, pulmonary artery sling and right aortic arch with vagus left subclavian artery in 2 cases, pulmonary artery sling and left aortic arch with vagus right subclavian artery in 1 case. In addition to 4 cases the remaining 38 patients were still combined with other cardiovascular malformations. Thirty-six cases of children underwent spiral CT airway remodeling, 23 children underwent fiber- bronchoscopy. In addition to 2 cases of airway abnormalities not seen, the rest of the children were present in varying degrees in different parts of tracheal stenosis or tracheomalacia. All patients underwent surgical correction of congenital vascular ring, concomitant heart deformity correction surgery according to the situation (7 cases of atrial septal defect repair, 3 of ventricular septal defect repair, 1 of mitral valvuloplasty, 1 of bi-Glenn, 1 of coarctation of the aorta correction with ventricular septal defect repair, 1 of trilogy of Fallot correction). One case suffered resection of tracheal stenosis and Slide tracheoplasty simultaneously, another case was implanted tracheal stenting postoperatively after pulmonary artery sling correction.

RESULTSThree patients died in hospital (7.1%), the cause of death were recurrent granuloma formation postoperatively. Remaining 39 patients were successfully discharged. The median time of overall survival of children with mechanical ventilation was 14 h (22 h) (M (Q(R))), median ICU residence time was 5 d (8 d), and the median in-hospital time was 19 d (9 d). Tracheal intubation time and postoperative ICU duration time in children with pulmonary artery sling children were much more than in children with double aortic arch (23 h (123 h) vs. 9 h(9 h), 7 d (13 d) vs. 4 d (2 d)), but the difference were not statistically significant. There was significant difference in the duration of hospitalization between the patient with the pulmonary artery sling and double aortic arch (23 d (9 d) vs. 16 d(6 d)) (χ(2) = 10.157, P = 0.006).

CONCLUSIONSThe recent results of surgical treatment of congenital vascular ring is safe and effective. The extent and scope of tracheal stenosis and tracheomalacia is a critical influence prognosis.

Aorta ; abnormalities ; Aorta, Thoracic ; abnormalities ; Child ; Child, Preschool ; Female ; Heart Defects, Congenital ; surgery ; Heart Septal Defects, Ventricular ; surgery ; Heart Ventricles ; abnormalities ; Humans ; Infant ; Infant, Newborn ; Male ; Prognosis ; Pulmonary Artery ; abnormalities ; Retrospective Studies ; Tomography, Spiral Computed ; Trachea ; surgery ; Tracheal Stenosis ; surgery ; Treatment Outcome