Medium and long-term follow-up of the Pul-Stent in treating postoperative branch pulmonary artery stenosis in children with congenital heart disease / 中华儿科杂志

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.

Transcatheter closure of patent ductus arteriosus with the new Amplatzer Duct OccluderII / 中华儿科杂志

<p><b>OBJECTIVE</b>To evaluate the indications, methodology and results of the transcatheter closure of patent ductus arteriosus (PDA) with the new Amplatzer Duct Occluder II (ADO-II).</p><p><b>METHOD</b>Totally 51 patients underwent transcatheter closure of PDA with the new ADO-II. The devices were delivered by 4F or 5F sheath through arterial or venous side respectively. The descending aorta angiography and transthoracic echocardiography was performed to evaluate the device position, residual shunt and complications caused by the device during and after implantation.</p><p><b>RESULT</b>Forty-nine patients had successful transcatheter closure of the PDA without significant residual shunts and artery obstruction during the short-term follow-up. One patient received the ADO-II dislodgment and first generation ADO re-implantation for the obvious descending aortal obstruction caused by ADO-II. Another patient had the ADO-II dislodgment and left pulmonary artery shaping surgery, because the ADO-II implantation led to obstruction of the left pulmonary artery. Both the obstructions in these two patients were ameliorated afterwards.</p><p><b>CONCLUSION</b>The transcatheter closure using the ADO-II is safe and effective for the non-window type PDA with a small size.</p>

Clinical presentation and therapeutic outcomes of carnitine deficiency-induced cardiomyopathy / 中华儿科杂志

<p><b>OBJECTIVE</b>Carnitine deficiency has been associated with progressive cardiomyopathy due to compromised energy metabolism. The objective of this study was to investigate clinical features of carnitine deficiency-induced cardiomyopathy and the therapeutic efficacy of L-carnitine administration.</p><p><b>METHOD</b>Between January 2010 and December 2011, filter-paper blood spots were collected from 75 children with cardiomyopathy. Free carnitine and acylcarnitine profiles were measured for each individual by tandem mass spectrometry (MS/MS). For those in whom carnitine deficiency was demonstrated, treatment was begun with L-carnitine at a dose of 150 - 250 mg/(kg·d). Clinical evaluation, including physical examination, electrocardiography, chest x-ray, echocardiography and tandem mass spectrometry, was performed before therapy and during follow-up.</p><p><b>RESULT</b>Of 75 cardiomyopathy patients, the diagnosis of carnitine deficiency was confirmed in 6 patients, which included 1 boy and 5 girls. Their age ranged from 0.75 to 6 years. Free carnitine content was (1.55 ± 0.61) µmol/L (reference range 10 - 60 µmol/L). Left ventricular end-diastolic diameter (LVDd) was (5.04 ± 0.66) cm and left ventricular ejection fraction (LVEF) was (38.5 ± 10.5)%. After 10 - 30 d therapy of L-carnitine, free carnitine content rose to (30.59 ± 15.02) µmol/L (t = 4.79, P < 0.01). LVDd decreased to (4.42 ± 0.67) cm (t = 4.28, P < 0.01) and LVEF increased to (49.1 ± 7.6)% (t = 6.59, P < 0.01). All patients received follow-up evaluations beyond 6 months of treatment. Clinical improvement was dramatic. LVEF returned to normal completely in all the 6 patients. LVDd decreased further in all the 6 patients and returned to normal levels in 3 patients. No clinical signs or symptoms were present in any of the 6 patients. The only complications of therapy had been intermittent diarrhea in 1 patient.</p><p><b>CONCLUSION</b>Tandem mass spectrometry is helpful to diagnose carnitine deficiency and should be performed in all children with cardiomyopathy. L-carnitine has a good therapeutic effect on carnitine deficiency-induced cardiomyopathy.</p>

Efficacy of the transcatheter closure of perimembranous and muscular ventricular septal defects with the Amplatzer duct occluder II / 中华心血管病杂志

<p><b>OBJECTIVES</b>To evaluate the feasibility and efficacy of transcatheter closure of perimembranous ventricular septal defects (pmVSD) with aneurysmatic formation and muscular ventricular septal defects (mVSD) with Amplatzer duct occluder II.</p><p><b>METHODS</b>This retrospective analysis included 48 cases received transcatheter closure of pmVSD aneurysmatic formation or mVSD from February 2011 to March 2012 in our hospital (42 pmVSD with aneurysmatic formation and 6 mVSD). Median age was 5.2 years (range: 1.8 - 15 years), and median weight was 20.2 kg (range: 12 - 44 kg). Amplatzer duct occluder II was selected depending on the condition of ventricular septal defect. The device was implanted by antegrade or retrograde approach. Complications such as residual shunt, valvular regurgitation and arrhythmia were evaluated by echocardiography or angiography. Median follow-up was 9.5 months (range: 1 - 13 months).</p><p><b>RESULTS</b>The mean ratio of pulmonary (Qp) to systemic (Qs) blood flow was 1.35 ± 0.15 before transcatheter closure. The diameter of exit hole of ventricular septal defects was (2.46 ± 0.53) mm measured by transthoracic echocardiography, and (2.35 ± 0.40) mm by angiography. Successful implantation of the device was achieved in 46 patients (96%) and unsuccessful in two cases due to acute aortic insufficiency. Forty-two (92%) patients were closed successfully, and trivial residual leak was evidenced in four patients and remained unchanged during follow-up. One patient with mVSD still had trivial residual shunt at 6 months post procedure. New trivial tricuspid insufficiency was observed in 1 patient (2.1%) during follow-up. Two patients developed procedural related left anterior fascicular block and remained unchanged during follow-up.</p><p><b>CONCLUSIONS</b>pmVSD with aneurysm and mVSD could be successfully treated with Amplatzer duct occluder II. However, the long waist and large disc of the device could interfere with tricuspid valve function and cause tricuspid insufficiency.</p>

Adenosine for pulmonary vasodilator testing in children with idiopathic pulmonary arterial hypertension / 中华儿科杂志

<p><b>OBJECTIVE</b>The assessment of pulmonary vascular reactivity plays an important role in the management of idiopathic pulmonary arterial hypertension (IPAH). The aim of this study was to explore the indications and methodology of pulmonary vasodilator testing in children with IPAH.</p><p><b>METHODS</b>From October 2009 to June 2011, a cohort of pediatric patients with IPAH in WHO functional classes II to III were enrolled in the study. Right heart catheterization was performed in all patients. After baseline hemodynamics were obtained, adenosine infusions were started at a dose of 50 µg/(kg·min), increased by 25 µg/(kg·min) at 2 min intervals to a maximum of 250 µg/(kg·min) or until a positive acute response.</p><p><b>RESULTS</b>A total of 15 patients with IPAH were enrolled in the study. The mean age of the patients was 6.3 yrs. Mean pulmonary artery pressure (mPAP) was (67.1 ± 15.9) mm Hg. Pulmonary capillary wedge pressure (PCWP) was (9.7 ± 2.9) mm Hg. Pulmonary vascular resistance index (PVRI) was (17.9 ± 7.5) Wood U·m(2). Three patients were responders, defined as a fall in mPAP of at least 10 mm Hg to a pressure level of 40 mm Hg or lower. Twelve patients were nonresponders according to the same criteria. Five out of the 15 patients experienced adverse effects, including chest discomfort (n = 1), systemic hypotension (n = 3) and bradycardia (n = 1). All side effects abated within 30-60 s of the discontinuation of the adenosine infusion.</p><p><b>CONCLUSION</b>Adenosine is an effective vasodilator in children with IPAH and can be used for safe and rapid assessment of vasodilator reserve in these patients.</p>

Evaluation of early monitoring of cardiotoxicity induced by anthracyclines / 中国当代儿科杂志

<p><b>OBJECTIVE</b>Anthracyclines (ANT) are effective for leukemia and solid tumors. However the long-term life quality of patients is seriously affected by ANT-related cardiotoxicity. The aim of this study was to evaluate the value of two dimension echocardiography (2DE) and serum biochemical indicators in monitoring ANT-related cardiotoxicity.</p><p><b>METHODS</b>Seventy children who received ANT chemotherapy (ANT dose: 124 ± 73 mg/m2) and were followed up for 22 ± 13 months were enrolled. 2DE with aspects of conventional indexes (left ventricular diameter and wall thickness, ejection fraction, E/A), myocardial performance index (MPI) and tissue Doppler imaging (TDI) were performed. Serum levels of troponin (CTnI) and brain natriuretic peptide (BNP) were measured. Thirty-seven healthy children served as the control group.</p><p><b>RESULTS</b>There were no significant differences in conventional indexes of 2DE between the ANT and the control groups. The MPI of left and right ventricular in the ANT group increased significantly compared with that in the control group (0.237 ± 0.06 vs 0.203 ± 0.06, 0.171 ± 0.05 vs 0.140 ± 0.04 respectively; P<0.01). TDI showed the late diastolic peak velocity in the basal and middle sections of left ventricular, interventricular septum and right ventricular in the ANT group were significantly higher than the controls. There were significant differences in the ratio of early to late diastolic peak velocity of the middle section of left ventricular and the basal and middle sections of the interventricular septum between the two groups (P<0.05). The changes of MPI and TDI became more obvious with the increased dose of ANT. There were no significant differences in serum CtnI and BNP levels between the two groups.</p><p><b>CONCLUSIONS</b>The heart function of patients who received ANT chemotherapy needs to be monitored for a long term. MPI and TDI can be used as early indexes for monitoring the heart function.</p>

Epidemiologic features of Kawasaki disease in Shanghai from 2003 through 2007 / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>The epidemiologic pictures of Kawasaki disease (KD) in Shanghai from 1998 through 2002 were reported, while the current status of KD in the following five years remains unknown.</p><p><b>METHODS</b>A questionnaire form and diagnostic guidelines for KD were sent to 50 hospitals providing pediatric medical care in Shanghai, China. All patients with KD diagnosed during January 2003 through December 2007 were recruited.</p><p><b>RESULTS</b>In total, 1187 cases of KD were enrolled. The incidence of KD was 36.78 to 53.28 (mean 46.32 ± 6.51) per 100 000 children under the age of 5 years between 2003 and 2007, which was higher than the year from 1998 to 2002 of (27.32 ± 7.11) per 100 000, (t = 4.406, P = 0.002). Ages at onset ranged from 12 days to 13.6 years (median 1.8 years). It occurred more frequently in summer and spring. Coronary arterial lesions (CAL), defined as ectasia or aneurysm, accounted for 19.8% (232 cases). Flattened or inverted T wave was the most frequent finding (194 cases, 20.5%) by electrocardiogram. Intravenous gamma-globulin was administrated to 1028 cases (86.6%). The occurrence of CAL seemed less frequent in the patients received gamma-globulin from day 5 to day 9 after the onset with the regimen of 1000 mg/kg once or 1000 mg/kg twice.</p><p><b>CONCLUSIONS</b>The incidence of KD was increasing in Shanghai. Treatment with intravenous gamma-globulin from day 5 to day 9 after the onset with the regimen of 1000 mg/kg once or 1000 mg/kg twice resulted in less coronary sequelae.</p>

Postoperative follow up of patients with complete atrioventricular septal defect / 中华心血管病杂志

<p><b>OBJECTIVE</b>To observe the operative efficacy of patients with complete atrioventricular septal defect (CAVSD).</p><p><b>METHODS</b>From January 2003 to June 2006, CAVSD patients underwent operative closure were included in this study. Color Doppler with apical four-chamber view was used to evaluate the degree of valve insufficiency before surgery and 2 days, 1 month, 6 months and 1 year after the surgery. Cardiac catheterization was performed to evaluate pulmonary artery pressure and pulmonary arteriolar resistance (PAR) before surgery in patients whose age were over 6 months. The time of staying at ICU, ventilation time after surgery and the occurrence of pulmonary artery hypertension crisis were recorded.</p><p><b>RESULTS</b>105 CAVSD patients underwent operative closure were enrolled in this study. The mean staying time at ICU was (4.7 +/- 2.4) days, and the mean ventilation time was (1.7 +/- 1.0)days, 9 patients (8.5%) developed pulmonary artery hypertension crisis after surgery. Patients with PAR > 8 Wood unit were older, staying time at ICU and ventilation time were longer compared patients with PAR < 8 Wood unit (all P < 0.05). The incidence of pulmonary artery hypertension crisis after surgery was also significantly higher in patients with PAR > 8 Wood unit compared patients with PAR < 8 Wood unit (P < 0.05). Hospital mortality was 3.8% (4/105). Three out of 4 hospital-dead patients had severe hypoplasia of the atrioventricular valve. Compared with preoperative, degree of valve insufficiency in both sides were relieved after surgery (P < 0.05). The valve insufficiency remained unchanged in 81 patients (77.1%), worsened in 14 patients (13.3%) and improved in 10 patients (9.6%) after surgery.</p><p><b>CONCLUSIONS</b>Our results suggested that early surgical repair for CAVSD was safe and beneficial. Preoperative PAR > 8 Wood unit was associated with increased risk of pulmonary artery hypertension crisis after surgery in patients with CAVSD.</p>

Therapeutic effect of Cheatham-Platinum stent implantation for vessel stenosis associated with congenital heart disease in children and adolescents / 中华儿科杂志

<p><b>OBJECTIVE</b>Although 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.</p><p><b>METHODS</b>From 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.</p><p><b>RESULTS</b>Totally 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.</p><p><b>CONCLUSION</b>Our 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.</p>

Noninvasive preoperative estimation of pulmonary vascular resistance of the patients waiting for two staged Fontan procedure / 中华儿科杂志

<p><b>OBJECTIVE</b>Noninvasive method for estimating the pulmonary vascular resistance (PVR) was used in patients waiting for two staged Fontan procedure to observe the relationship between estimated PVR and surgical results.</p><p><b>METHODS</b>Thirty-three candidate patients for two staged Fontan procedures were randomly selected for this trial. Preoperative hemoglobin (HGB), hematocrit (HCT) and pulse oxygen saturation (SpO2) were measured. Estimated PVRs were then calculated by the regression equations. The cases were divided into three groups of low risk group with PVR less than 2.4 wood, high risk group with PVR between 2.4 to 3.2 wood, and extremely high risk group with PVR more than 3.2 wood. The incidences of postoperative low cardiac output and multi-organ failure were compared. Simultaneously, the cases without or with postoperative severe low cardiac output and multi-organ failure after operation were divided into two groups. The preoperative HGB, HCT and estimated PVR among the groups were analyzed.</p><p><b>RESULTS</b>The rates of postoperative severe low cardiac output and multi-organ failure were 10.0%, 32.4% and 63.6% for the patients of low, high and extremely high risk groups respectively. The difference was significant (P < 0.01). The preoperative HGB, HCT, SpO2 and PVR were all significantly different between the patients without or with postoperative severe low cardiac output and multi-organ failure. The PVR (HGB) were (2.53 +/- 0.56) wood and (3.24 +/- 0.58) wood respectively. The PVR (HCT) were (2.59 +/- 0.58) wood and (3.21 +/- 0.79) wood respectively. The PVR (SpO2) were (2.22 +/- 0.55) wood and (2.93 +/- 0.58) wood, respectively. The differences were all significant (t = 3.25, 2.52 and 3.33 respectively, P < 0.01 or 0.05).</p><p><b>CONCLUSIONS</b>Preoperative estimated PVRs calculated by HGB, HCT and SpO2 were comparable with the postoperative results. Thus, the invasive preoperative estimation of pulmonary vascular resistance could be a method in estimating the surgical indication and predicting the surgical result for two stage Fontan procedure.</p>

A novel mutation in the BMPR2 gene in familial pulmonary arterial hypertension / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Familial pulmonary arterial hypertension (FPAH) is an autosomal dominant disorder characterized by plexiform lesions of endothelial cells in pulmonary arterioles which leads to elevated pulmonary arterial pressure, right-sided heart failure and death. Heterozygous mutations in the bone morphogenetic protein type II receptor gene (BMPR2) have been found to underlie a majority of FPAH cases. More than 140 distinct mutations have been identified in FPAH cases and in idiopathic pulmonary arterial hypertension (IPAH) cases, but only one mutation has been reported in Chinese patients.</p><p><b>METHODS</b>A three-generation pedigree of FPAH and another 10 patients with IPAH were collected. In the family, two of the 9 surviving and one deceased family member were diagnosed as FPAH. The entire protein-coding region and intron/exon boundaries of the BMPR2 gene were amplified by PCR using DNA samples from affected individuals. Direct sequencing of PCR products was performed on both the sense and antisense strands. To confirm the segregation of the mutation within the family and exclude the presence of the mutation in normal subjects, the relevant exon was amplified by PCR, followed by mutation-specific RPLP analysis.</p><p><b>RESULTS</b>In the Chinese pedigree with FPAH an A-to-T transition at position 1157 in exon 9 of the BMPR2 gene was identified which resulted in a Glu386Val mutation. We confirmed the segregation of the mutation within the family and excluded the presence of the mutation in a panel of 200 chromosomes from normal subjects. No mutation was detected in BMPR2 in the other 10 patients with IPAH.</p><p><b>CONCLUSIONS</b>This amino acid substitution occurs at a glutamic acid that is highly conserved in all type II TGF-beta receptors. The nearly invariant Glu forms an ion pair with an invariant Arg at position 491 thereby helping to stabilize the large lobe. Substitution of Arg at position 491 is the most frequently observed missense mutation in FPAH, but until now no mutations at position 386 have been found in FPAH. The predicted functional impact of the Glu386Val mutation and its absence in healthy controls support the mutation as the cause of FPAH.</p>

Anomalous origin of the left coronary artery from the pulmonary artery: report on 10 cases / 中国当代儿科杂志

<p><b>OBJECTIVE</b>The anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary artery abnormality. This study aimed to investigate the clinical characteristics, diagnosis and treatment of the disorder.</p><p><b>METHODS</b>The medical data, including common clinical presentations, the findings of the digital subtraction angiogram (DSA), echocardiograms and the electrocardiograph (ECG), surgical records and the outcome, of 10 children with ALCAPA between June 2001 and February 2005 were retrospectively reviewed.</p><p><b>RESULTS</b>Diaphoresis, fantod, breathlessness and difficult feeding were commonly presented in the patients with ALCAPA. ECG, echocardiography and DSA examinations showed characteristic changes in these patients. The Takeuchi procedure was performed in 4 patients, direct aortic reimplantation in 4 cases and aortic reconstruction in 2 cases. After operation the symptoms of 8 patients were improved but 2 died.</p><p><b>CONCLUSIONS</b>ALCAPA patients have characteristic clinical manifestations and lab findings. This is helpful in making an early diagnosis of ALCAPA. Early surgical treatment can improve the patients' outcome.</p>

Exercise capacity evaluation after percutaneous balloon pulmonary valvuloplasty in children with pulmonary valve stenosis / 中华心血管病杂志

<p><b>OBJECTIVE</b>To assess exercise capacity after percutaneous balloon pulmonary valvuloplasty (PBPV) in children with isolated pulmonary valve stenosis.</p><p><b>METHODS</b>Treadmill 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.</p><p><b>RESULTS</b>Post 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.</p><p><b>CONCLUSION</b>Exercise capacity post percutaneous balloon pulmonary valvuloplasty in children with isolated pulmonary valve stenosis was comparable to that in healthy children.</p>

Evaluation of the development of pulmonary vessels with pulmonary venous index in congenital heart disease with decreased pulmonary blood flow / 中华儿科杂志

<p><b>OBJECTIVE</b>The development status of pulmonary artery is one of the most important criteria for decision-making strategy and predicting postoperative outcome in congenital heart disease with decreased pulmonary blood flow. Currently, Nakata index and McGoon index have been used as morphologic index in evaluating the development status of pulmonary artery. Those indices have some shortcoming. It was recently found that pulmonary veins index is a more precise morphological indicator of pulmonary blood flow and development status of pulmonary vessels. This study aimed to explore an index of evaluating pulmonary blood stream and the development of pulmonary vessels, as a criterion for surgical decision-making strategy.</p><p><b>METHODS</b>The diameters of left and right pulmonary arteries and pulmonary veins were measured on DSA films in 74 patients with congenital heart disease with decreased pulmonary blood flow, The correlative analysis was done between Nakata index, McGoon index, pulmonary vein index (PVI) and postoperative outcome which were the length of stay in ICU, duration of mechanical ventilation and dose of inotropic drugs.</p><p><b>RESULTS</b>Excellent correlations between the size of pulmonary veins and pulmonary arteries were found, the correlation between left pulmonary veins and distal portion of left pulmonary artery was 0.73, between left pulmonary veins and proximal portion of left pulmonary artery was 0.72, right pulmonary veins and distal portion of right pulmonary artery was 0.67, and right pulmonary veins and proximal portion of right pulmonary artery was 0.71. The length of stay in ICU, duration of mechanical ventilation and dose of inotropic drugs correlated well with PVI (r = -0.51, -0.478, and -0.693). Compared with Nakata index and McGoon index, PVI was a better criterion for evaluating the developmental status of the whole pulmonary vessels. In the right ventricular outlet reconstruction patients, the McGoon index for patients with low cardiac output syndrome (LCOS) was 1.36 +/- 0.51, and 1.97 +/- 0.58 for patients without LCOS (t = 2.347, P < 0.05), the Nakata index for patients with LCOS was 164 +/- 106 mm(2)/m(2) and 269 +/- 124 mm(2)/m(2) for patients without LCOS (t = 2.218, P < 0.05), the PVI for patients with LCOS was 152 +/- 77 mm(2)/m(2) and 273 +/- 125 mm(2)/m(2) for patients without LCOS (t = 2.936, P < 0.01), pulmonary vessel index of patients with LCOS was less than that of those without LCOS. When PVI was < or = 180 mm(2)/m(2), postoperative hemodynamics was unstable, the frequency of low cardiac output syndrome and mortality significantly increased.</p><p><b>CONCLUSIONS</b>The development of pulmonary arteries and pulmonary veins correlated with each other. PVI is a precise morphological indicator of pulmonary blood flow and development of pulmonary vessels. It is a helpful indicator to decide surgical strategy.</p>

Congenital coronary artery fistula in children:The interventional management and outcome / 介入放射学杂志

Objective To assess the safety and efficacy of transcatheter closure of congenital coronary artery fistulas(CAFs).Methods Retrospective analysis was performed on 19 patients mean age of(5.5?4.1) years treated from February 1995 to December 2005 with transcatheter closure of CAFs using transcatheter spring coil embolization,Amplatzer PDA occluder or Amplatzer plug.One case had a residul fistula postoperatively associated with patent duetus arteriosus(PDA).Results The abnormal parameters included mean fistula diameter(3.7?1.6)mm(2.5-8.2 mm),pulmonary mean pressure(28.0?5.0)mmHg(25.0-67.0 mmHg)and pulmonary to systemic shunt(Qp/Qs)1,6?0.8(1.0-2.3).The sites of the fistulas were originated in right coronary artery 11,left anterior descending coronary artery or left circumflex coronary artery 8. Abnormal communication sites of these fistulas were to right ventricle in 14 and right atrium in 5.Various occlusion devices used to close these fistulas included one Giantureo coil in 10,2-4 Gianturco coils in 3, Duct-Occlud in 3,Amplatzer duct occluder in 2 and Amplatzer plug in 1.The post-operative residul fistula with PDA was treated successfully with PDA occlusion.The immediate,one month and one year complete occlusion rates were 55.6%(10/18),88.9%(16/18),100%(18/18),respectively.The coil slipped into the left pulmonary artery in 1 case and correction was obtained by retrieving with forceps.Follow-up studies at 3 months to 4.3 years showed complete abolition of shunt in all patients with no evidence of recanalization leading to recurrences of shunt.Conclusion Transcatheter closure of CAFs is a safe and effective alternative to surgical repair.

Diagnostic value of the currently used criteria and brain natriuretic peptide for diagnosing congestive heart failure in children with congenital heart disease / 中华儿科杂志

<p><b>OBJECTIVE</b>To improve the accuracy of diagnosis of heart failure (HF) has been the focus of research for a long time. The diagnosis for HF with congenital heart disease, however, is more difficult. The aim of the study was to evaluate the diagnostic criteria for HF in children and examine the value of plasma brain natriuretic peptide (BNP) and NT-proBNP for diagnosing HF in pediatric patients with congenital heart disease, and to look for the most valuable index for the diagnosis according to the multifactor analysis.</p><p><b>METHODS</b>Totally 118 children with congenital heart disease were enrolled. They were diagnosed using modified Ross score, Qingdao criteria, NYU PHFI, and plasma BNP and NT-proBNP. According to modified Ross score as the referent criteria, other diagnostic criteria and plasma BNP and NT-proBNP were studied. The sensitivity, specificity and area of the ROC curve were examined. Logistic regression analysis was used to select the valuable index for diagnosing HF.</p><p><b>RESULTS</b>(1) The value of each clinical criteria: 1 The sensitivity of Qingdao criteria for diagnosing HF was 47.9%. The specificity was 100% and the accuracy was 57.6%. 2 There were 52 patients younger than six months in whom 27 (51.9%) were breast fed. Only 25 children were measured with Ross score. The Ross score was positively correlated with the modified Ross score (r = 0.948). The area under the ROC curve of Ross score diagnosing HF was 0.985, and the sensitivity was 88%, while the specificity was 100%. 3 NYU PHFI score was positively correlated with the modified Ross score. The area under the ROC curve of the NYU PHFI diagnosing HF was 0.964, and the sum of sensitivity and specificity was favorite when > or = 8 was set as the cut-off point. If > 2 was set as cut-off point, it had a high sensitivity but a low specificity. The sensitivity of NYU PHFI was 100% > was set 2 as cut-point for diagnosing HF, but the specificity was 4.5%. (2) Plasma BNP and NT-proBNP were positively correlated with the modified Ross score, and increased with the severity of congestive HF. The area under the ROC curve of BNP was 0.880, and the cut-off line was > or = 349 pg/ml. The area under the ROC curve of NT-proBNP was 0.981, and the cut-off line was > or = 499 fmol/ml. (3) Logistic regression analysis showed that in multifactor analysis, only plasma concentration of NT-proBNP, dyspnea, tachycardia, tachypnea, failure to thrive were the independent predictors for diagnosing HF. (4) Plasma concentration of NT-proBNP incorporated with clinical criteria would improve its accuracy.</p><p><b>CONCLUSION</b>All the clinical criteria commonly used were valuable for diagnosing HF in children with congenital heart disease, but each has its own limits, such as the low sensitivity of Qingdao, the low adaptation of Ross score because of the high breast-feeding rate in our country and the low specificity of NYU PHFI when > 2 was set as the cut-off point. Plasma concentrations of BNP and NT-proBNP were valuable for diagnosing HF in children with congenital heart disease, and NT-proBNP was the independent predictor for HF.</p>

Risk factors for arrhythmia early after transcatheter closure of perimembranous ventricular septal defects / 中华儿科杂志

<p><b>OBJECTIVE</b>Perimembranous ventricular septal defects (PMVSDs) is the most common type of congenital ventricular septal defects (VSD), which accounts for 70% approximately 80% of VSD. The structure of PMVSDs is very complex, it is close to tricuspid valve, mitral valve and aortic valve. The atrioventricular (AV) node is located in the posterior upper membranous ventricular septum and branches into left and right bundle in the posterior lower margin. This increases the risk of transcatheter closure of PMVSDs. Arrhythmias is the common complication after transcatheter closure of PMVSDs. The present study aimed to identify the risk factors resulting in arrhythmias after transcatheter closure of PMVSDs in patients under 18 years of age to decrease the incidence of arrhythmias after the interventional catheterization.</p><p><b>METHODS</b>A retrospective analysis was performed on the patients treated with transcatheter intervention from June 2002 to June 2004. Transcatheter closure of PMVSDs with Amplatzer membranous septal occluder and a domestic product was performed in 89 cases after obtaining consent themselves and/or their guardian or parents, 47 cases were males and 42 females. The age of the cases ranged from 3 to 18 years (mean 8.2 years) and the body weight ranged from 13 to 55 kg (mean 26.7 kg). They were all diagnosed as having PMVSDs with trans-thoracic echocardiography (TTE) before the interventional catheterization, the electrocardiographic (ECG) and chest X-ray (CXR) findings were recorded. A simultaneous care ECG and TTE were performed during operation in order to identify the effect of the transcatheter closure, the heart structure and functional changes and whether or not arrhythmias occurred, respectively. In 80 cases AGA Amplatzer membranous septal occluder was used and in 9 cases a domestic product was used. Follow-up was performed based on the echocardiography and ECG.</p><p><b>RESULTS</b>The devices were successfully implanted in 89 cases; 11 cases (12%) developed various block of heart conduction within 5 days, which included first degree AV block in 1 patient, third degree AV block in 1, left anterior bundle branch block in 5, partial right bundle branch block in 4, complete right bundle branch block in 3, and 3 patients had two kinds of heart block. Eight patients were treated with corticosteroids, 6 of them recovered within 14 days, 1 patient within 1 month and in 1 case the problem shifted from first degree block and left anterior bundle branch block to left anterior bundle branch block 5 days later and that persisted for 6 months. It was found that the distance from upper margin of defects to the aortic valve < 3 mm, the diameter of ventricular septal defect > or = 8 mm, the diameter of device > or = 10 mm, blood pH < 7.35 and arteriovenous track building time after the success of the Seldinger technique > or = 60 min were independent predictors of post-closure arrhythmias.</p><p><b>CONCLUSION</b>Arrhythmias remain the severe early complications after interventional catheterization for PMVSDs in patients under 18 years of age. Shortening of operation time, prevention of acidosis and strict selection of indications may be the most effective measures to prevent arrhythmias after transcatheter closure of PMVSDs in patients under 18 years of age.</p>

Diagnostic value of plasma concentration of pro-brain natriuretic peptide in congestive heart failure in pediatric patients with ventricular septal defects / 中华儿科杂志

<p><b>OBJECTIVE</b>The value of plasma brain natriuretic peptide (BNP) and N-terminal proBNP (NT-proBNP) can reflect cardiac function and therefore can be used for diagnosing congestive heart failure (CHF) and evaluating cardiac function. There are few reports, however, on the value of BNP and NT-proBNP in pediatric cases of congenital heart defect. The aim of this study was to assess the value of plasma NT-proBNP in the diagnosis of CHF and evaluation of cardiac function in pediatric patients with ventricular septal defect (VSD).</p><p><b>METHODS</b>Fifty-one patients with VSD aged from 2 months to 2 years old (mean 7.9 months) were enrolled. According to the modified Ross Score, the patients were divided into three groups, no CHF group (20 patients), mild CHF group (18 patients) and moderate to severe CHF group (13 patients). Fifteen age-matched normal children were used as controls. Plasma NT-proBNP was measured using enzyme immunoassay. All patients had complete echocardiographic study, including measurement of left ventricular end diastolic volume index (LVEDVI), left ventricular end systolic wall stress (LVSEWS), heart rate corrected mean velocity of circumferential fiber shortening (mVcFc), left ventricular ejection fraction (LVEF), left ventricular fractional shortening (LVFS), and contractility index (Con). The correlation between plasma NT-proBNP level and modified Ross Score and echocardiographic cardiac functional indexes was determined. The sensitivity, specificity and ROC curve of plasma NT-proBNP for diagnosing CHF was studied.</p><p><b>RESULTS</b>Plasma NT-proBNP was positively correlated with modified Ross Score (r = 0.75, P < 0.01). Plasma NT-proBNP concentration in moderate to severe CHF group (2061 +/- 908) fmol/ml was significantly higher than that of mild CHF group (810 +/- 335) fmol/ml, and Plasma NT-proBNP concentration in mild CHF group was higher than that in no CHF group (309 +/- 68) fmol/ml. 97.14% of normal controls and subjects in no CHF group had their plasma NT-proBNP below 400 fmol/ml. 83.3% of children in mild CHF group had their plasma NT-proBNP between (400-1400) fmol/ml while in moderate and severe CHF group 84.6% of children had their plasma NT-proBNP beyond 1400 fmol/ml. Plasma NT-proBNP was also positively correlated with LVEDVI and LVSEWS. There was no correlation among mVcFc, LVEF, LVFS, Con and plasma NT-proBNP concentration. Using plasma NT-proBNP concentration > or = 400 fmol/ml as cut-point for diagnosing CHF, the sensitivity was 89.3%, the specificity was 91.2%, and the area under the ROC curve was 0.944.</p><p><b>CONCLUSIONS</b>Plasma NT-proBNP level could be used to assess cardiac function and diagnose CHF in pediatric patients with VSD.</p>

Interventional therapy for complex congenital heart disease / 中华儿科杂志

<p><b>OBJECTIVE</b>The 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.</p><p><b>METHODS</b>Balloon 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.</p><p><b>RESULTS</b>In 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.</p><p><b>CONCLUSION</b>In management of complex congenital heart diseases, combination of surgical procedure and interventional catheterization therapy could be suggested to have better outcome.</p>

Arrhythmia in the long-term follow-up after intracardiac repair of tetralogy of Fallot / 中华儿科杂志

<p><b>OBJECTIVE</b>The long-term success of intracardiac repair of tetralogy of Fallot is hampered by the occurrence of arrhythmias. The aim of the present study was to determine the incidence of arrhythmias after intracardiac repair of tetralogy of Fallot and their correlation with surgical and clinical findings.</p><p><b>METHODS</b>The study group consisted of 54 patients, 35 males and 19 females. They underwent repair at a mean age of 51 months (range 17 to 117 months). The median age at the time of study was 9.4 years (range 5 to 14 years), and the mean duration of follow-up was 4.3 years (range 2.8 to 9.0 years) after surgery. The follow-up study included routine ECG, 24 hour Holter, echocardiography and exercise testing.</p><p><b>RESULTS</b>The incidence of ventricular arrhythmia was 33 percent, and 1 patient had non-sustained ventricular tachycardia. All patients with elevated right ventricular pressure had ventricular arrhythmias, and 28% of patients with normal right ventricular pressure had ventricular arrhythmias (P < 0.05). 53% of patients had significant ventricular arrhythmias when the duration of cardiopulmonary bypass was > or = 90 minutes, as opposed to 23% when it was < 90 minutes (P < 0.05). There was no significant difference in prevalence of ventricular arrhythmias between mild pulmonary regurgitation and severe pulmonary regurgitation (21% vs 40%, P > 0.05). No significant difference was found in the incidence of ventricular arrhythmias between follow-up in five years and more than five years (32% vs 34%, P > 0.05). Age at surgery correlated with the prevalence of ventricular arrhythmias (r = -0.221, P < 0.05). Eight patients (15%) had supraventricular arrhythmias.</p><p><b>CONCLUSION</b>The frequency of ventricular arrhythmias correlated with elevated right ventricular systolic pressure, the duration of cardiopulmonary bypass, and the age at surgery. No correlation was found between pulmonary regurgitation and the duration of follow-up. Exercise-induced frequent multiform premature ventricular complexes were associated with abnormal hemodynamic status and high risk of ventricular tachycardia.</p>