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Objective:To investigate the clinical manifestations, diagnosis, treatment and prognosis of Kawasaki disease (KD) complicated with acute abdomen in children.Methods:Clinical manifestations, laboratory examinations, abdominal B-ultrasound scans, abdominal plain films, abdominal CT findings, coronary artery lesions and prognosis of 16 children with KD and acute abdomen admitted to Guangzhou Women and Children′s Medical Center, Guangzhou Medical University from August 1, 2015 to July 31, 2019 were retrospectively analyzed.The measurement data of age, leukocyte, hemoglobin, platelet and biochemical indexes are expressed by M (range). Results:A total of 16 cases were included, involving 7 males and 9 females aged 4 years and 8 months (7 months to 8 years). Among them, 9 cases of KD complicated with cholecystitis, 5 cases with intestinal obstruction, 2 cases with acute appendicitis, 2 cases with necrotizing enterocolitis, 2 cases with acute peritonitis and 1 case with acute pancreatitis.There were 3 cases complica-ted with 2 or more kinds of acute abdomen diseases.All 16 patients had symptoms of abdominal pain, 7 cases had vomiting, 4 cases had obvious abdominal distension and 1 case had bloody stool.Abdominal B-ultrasound was performed in all cases, and 8 cases showed enlargement of gallbladder and edema and thickening of gallbladder wall, 2 cases of appendicitis, 2 cases of intestinal obstruction and 1 case of pancreatic enlargement.Abdominal plain film examination was performed in 8 cases, suggesting necrotizing enterocolitis in 2 cases and intestinal obstruction in 2 cases.Abdominal CT examination was performed in 3 cases, including edema of gallbladder wall in 1 case, peritonitis in 1 case, intestinal obstruction in 2 cases and enlargement of pancreas in 1 case.All the 16 cases were treated with pulsed intravenous immunoglobulin (IVIG) at 2 g/kg and antiplatelet agents.Eight IVIG-resistant cases were treated with the second dose of IVIG at 2 g/kg, among whom, 4 children still had fever and intravenous Methylprednisone was given.Two cases underwent enterostomy and abdominal puncture drainage.All the 16 children were followed up until 6 months after discharge, and 4 cases (25%) were complicated with coronary artery aneurysm (CAA) during the acute stage or follow-up period.Conclusions:KD complicated with acute abdomen is more commonly manifested as cholecystitis and intestinal obstruction.Besides the classic symptoms of KD, abdominal pain and vomiting are the most common in KD with acute abdomen.Abdominal ultrasound, plain film and CT examinations are conductive to the early diagnosis of KD complicated with acute abdomen.In addition, the incidence of IVIG-resistance and CAA is relatively high in children with KD complicated with acute abdomen.
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Objective@#To investigate the characteristics of long-term changes in giant coronary artery aneurysm (GCAA) caused by Kawasaki disease (KD) and the technical approach of using selective coronary angiography (SCAG) in children.@*Methods@#A retrospective analysis was made in 52 patients with KD and GCAA in acute phase, including 38 males and 14 females with an average age of (4.1±2.5) years old ranging from 1 to 14 years old, from January 2008 to December 2018 at the Department of Cardiology, Guangzhou Women and Children′s Medical Center.The selective coronary angiography (SCAG) was performed in the patients who were followed up for (10.6±3.6) years (1-19 years). The changes in coronary artery lesions were analyzed and the technical approach of SCAG was discussed in children.@*Results@#Among 52 patients investigated by SCAG, the location of coronary artery lesion was found the left-anterior descending branch in 21 cases(40.4%), 20 cases(38.5%) in the right coronary artery, 8 cases(15.4%) in the left main trunk and 3 cases(5.7%) in circumflex.The left coronary artery in 2 cases and the right coronary artery in 4 cases were completely occluded, and collateral vessel formation was found in 12 cases.There were 21 cases of right coronary artery stenosis, including 7 cases of the right coronary artery occlusion and bridge-like neovascularization, and 4 cases of the right coronary artery woven lesions (thrombotic occlusion and clustered neovascularization). There were 27 cases of the left coronary artery stenosis with different degrees, including 5 cases of the left coronary artery occlusion and bridge-like neovascularization, and 2 cases of the left coronary artery woven lesions (thrombotic occlusion and clustered neovascularization). All of 52 patients with GCAA were followed up for 1 to 19 years.GCAA still existed in 40 cases.Regression to small coronary artery aneurysm was found in 8 cases, and mild-dilation regression was observed only in 4 patients.The resting electrocardiogram showed pathological Q wave and the left ventricular enlargement in 6 cases, and normal in the remaining 46 patients.The children had no subjective symptoms and their activities were not restricted.Compared with SCAG, echocardiography detected 52 cases with proximal morphological changes in the coronary artery, but distal changes were found only in 3 cases.And 12 cases with coronary artery occlusion and neovascularization failed to be prompted by echocardiography.@*Conclusions@#GCAA induced by KD can persist for a long time, and has characteristics of coronary occlusion, stenosis, and collateral vessel formation.The conventional electrocardiogram and echocardiography are insensitive to the coronary artery morphology and dysfunction in KD sequelae.The SCAG is of great value in tracking this disease.For using SCAG in children, as long as the approp-riate equipment and projection angle are selected, and the operation skills are mastered, satisfactory images and high success rate can be obtained.
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Objective@#To investigate the safety and efficacy of Warfarin combined with Aspirin in the treatment of multiple medium and giant coronary artery aneurysms in Kawasaki disease(KD).@*Methods@#Clinical and follow-up data of 45 children diagnosed with KD complicated with multiple medium-sized and giant coronary artery aneurysms from April 2014 to December 2018 at Guangzhou Women and Children′s Medical Center were collected.These children were divided into 2 groups.A total of 31 cases received regular oral Warfarin combined with Aspirin called experimental group.There were 14 patients treated with oral Aspirin and Clopidogrel called control group.General information, laboratory examination, electrocardiogram, echocardiography, outcome and bleeding complications of the 2 groups were analyzed retrospectively.@*Results@#(1) In experimental group, there were 22 patients found thrombosis under echocardiography.The 10 patients′ thrombosis disappeared, 5 patients′ thrombosis reduced, and 2 patients′ increased after treatment.In control group, there were 5 cases found thrombosis.The 2 cases′ thrombosis reduced and 3 cases′ throm-bosis increased.The number of thrombosis in experimental group was significantly reduced, and the number of new thrombosis was less than that in control group (χ2=6.454, P<0.05). (2) The number of coronary artery aneurysms in experimental group increased slowly than that in control group [12.90%(4/31 cases)vs.14.28%(2/14 cases)]. (3) The number of coronary artery aneurysms in experimental group decreased rapidly than that in control group [23.91%(11/46 cases)vs.10.00%(1/10 cases)]. (4) The number of cases of tumor retraction in experimental group was more than that in control group [74.19%(23/31 cases)vs.42.85%(6/14 cases)]. (5) During the followed-up, there was no abnormality in the blood phosphokinase isozyme and troponin, no abnormality in the electrocardiogram and echocardiogram, no ventricular enlargement and abnormal ventricular wall movement, and the ejection fraction value was within the normal range.No active bleeding and no death occurred in the two groups.@*Conclusions@#Warfarin combined with Aspirin is very safe and effective in the treatment of KD coronary tumor, it can reduce thrombosis effectively.Compared with oral Aspirin and Clopidogrel, Warfarin combined with Aspirin can reduce the number of multiple medium-sized and large coronary artery aneurysms and reduce the diameter of coronary artery aneurysms.
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Objective@#To summarize the clinical characteristics of pediatric hypertrophic cardiomyopathy and analyze its etiology for providing guidance for early identification, diagnosis and prognosis.@*Methods@#Fifty-two cases of pediatric hypertrophic cardiomyopathy admitted to Guangzhou Women and Children′s Medical Center from January 2012 to June 2018 were retrospectively analyzed and summarized from the aspects of age, gender, family history, clinical features, auxiliary examination, etiology, drug efficacy and disease outcome.@*Results@#(1) There were 52 cases in this group including 36 males and 16 females.The ages of patients ranged from 15 days to 14 years (with mean age of 27.7 months, median 6.5 months). A total of 34 patients (65.4%) were followed up for 1-78 months (mean 30.6 months). Echocardiography showed 52 cases of left ventricular wall thickening (100%), 21 cases of double ventricular hypertrophy (40.4%), 18 cases of left ventricular outflow tract obstruction (34.6%), and 18 cases of hepatic enzyme elevation (34.6%). The etiology of 11 cases was clear (21.2%), including 7 cases of type Ⅱ glycogen accumulation, 3 cases of Noonan syndrome and 1 case of primary carnation deficiency.No routine heart transplantation was performed at the end of follow-up, and 12 patients (35.3%) died, 7 cases of whom died in infancy.@*Conclusions@#Children with hypertrophic cardiomyopathy have a relatively young age, so it is necessary to search for the etiology actively, carry out disease risk assessment, and conduct personalized management and treatment.
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Objective To investigate the characteristics of long-term changes in giant coronary artery aneurysm (GCAA) caused by Kawasaki disease (KD) and the technical approach of using selective coronary angiography (SCAG) in children.Methods A retrospective analysis was made in 52 patients with KD and GCAA in acute phase,including 38 males and 14 females with an average age of (4.1 ±2.5) years old ranging from 1 to 14 years old,from January 2008 to December 2018 at the Department of Cardiology,Guangzhou Women and Children's Medical Center.The selective coronary angiography (SCAG) was performed in the patients who were followed up for (10.6 ± 3.6) years (1-19 years).The changes in coronary artery lesions were analyzed and the technical approach of SCAG was discussed in children.Results Among 52 patients investigated by SCAG,the location of coronary artery lesion was found the left-anterior descending branch in 21 cases(40.4％),20 cases(38.5％) in the right coronary artery,8 cases (15.4％) in the left main trunk and 3 cases(5.7％) in cimumflex.The left coronary artery in 2 cases and the right coronary artery in 4 cases were completely occluded,and collateral vessel formation was found in 12 cases.There were 21 cases of right coronary artery stenosis,including 7 cases of the right coronary artery occlusion and bridge-like neovascularization,and 4 cases of the right coronary artery woven lesions (thrombotic occlusion and clustered neovascularization).There were 27 cases of the left coronary artery stenosis with different degrees,including 5 cases of the left coronary artery occlusion and bridge-like neovascularization,and 2 cases of the left coronary artery woven lesions (thrombotic occlusion and clustered neovascularization).All of 52 patients with GCAA were followed up for 1 to 19 years.GCAA still existed in 40 cases.Regression to small coronary artery aneurysm was found in 8 cases,and mild-dilation regression was observed only in 4 patients.The resting electrocardiogram showed pathological Q wave and the left ventricular enlargement in 6 cases,and normal in the remaining 46 patients.The children had no subjective symptoms and their activities were not restricted.Compared with SCAG,echocardiography detected 52 cases with proximal morphological changes in the coronary artery,but distal changes were found only in 3 cases.And 12 cases with coronary artery occlusion and neovascularization failed to be prompted by echocardiography.Conclusions GCAA induced by KD can persist for a long time,and has characteristics of coronary occlusion,stenosis,and collateral vessel formation.The conventional electrocardiogram and echocardiography are insensitive to the coronary artery morphology and dysfunction in KD sequelae.The SCAG is of great value in tracking this disease.For using SCAG in children,as long as the appropriate equipment and projection angle are selected,and the operation skills are mastered,satisfactory images and high success rate can be obtained.
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Objective To investigate the safety and efficacy of Warfarin combined with Aspirin in the treatment of multiple medium and giant coronary artery aneurysms in Kawasaki disease(KD).Methods Clinical and followup data of 45 children diagnosed with KD complicated with multiple medium-sized and giant coronary artery aneurysms from April 2014 to December 2018 at Guangzhou Women and Children's Medical Center were collected.These children were divided into 2 groups.A total of 31 cases received regular oral Warfarin combined with Aspirin called experimental group.There were 14 patients treated with oral Aspirin and Clopidogrel called control group.General information,laboratory examination,electrocardiogram,echocardiography,outcome and bleeding complications of the 2 groups were analyzed retrospectively.Results (1) In experimental group,there were 22 patients found thrombosis under echocardiography.The 10 patients' thrombosis disappeared,5 patients' thrombosis reduced,and 2 patients' increased after treatment.In control group,there were 5 cases found thrombosis.The 2 cases' thrombosis reduced and 3 cases' thrombosis increased.The number of thrombosis in experimental group was significantly reduced,and the number of new thrombosis was less than that in control group (x2 =6.454,P ＜ 0.05).(2) The number of coronary artery aneurysms in experimental group increased slowly than that in control group [12.90％ (4/31 cases) vs.14.28％ (2/14 cases)].(3) The number of coronary artery aneurysms in experimental group decreased rapidly than that in control group [23.91％ (11/46 cases)vs.10.00％ (1/10 cases)].(4) The number of cases of tumor retraction in experimental group was more than that in control group [74.19％ (23/31 cases)vs.42.85％ (6/14 cases)].(5) During the followed-up,there was no abnormality in the blood phosphokinase isozyme and troponin,no abnormality in the electrocardiogram and echocardiogram,no ventricular enlargement and abnormal ventricular wall movement,and the ejection fraction value was within the normal range.No active bleeding and no death occurred in the two groups.Conclusions Warfarin combined with Aspirin is very safe and effective in the treatment of KD coronary tumor,it can reduce thrombosis effectively.Compared with oral Aspirin and Clopidogrel,Warfarin combined with Aspirin can reduce the number of multiple medium sized and large coronary artery aneurysms and reduce the diameter of coronary artery aneurysms.
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Objective To analyze the treatment and follow-up of congenital coronary artery fistula (CAF) with giant coronary artery aneurysm (GCAA) in children.Methods The clinical data were analyzed retrospectively in 13 patients who were diagnosed as congenital CAF with GCAA between July 2009 and December 2016 in Guangzhou Women and Children's Medical Center.There were 8 boys and 5 girls.The median age was 18 months,ranging from 40 days to 12 years old.The body weight ranged from 3.8 kg to 29.0 kg with a median of 8.8 kg.Fistulas originated from right coronary artery accounted in 8 patients,with 5 from left coronary artery.Fistulas drained into right atrium in 3 patients,right ventricular in 8 patients and left ventricular in 2 patients.Single fistula occurred in 12 patients and multiple fistulas in 1 patient.The diameter of coronary artery aneurysm ranged from 8 mm to 16 mm with a median of 9.2 mm.Results One patient had tachypnea and growth retardation without heart murmur.The other 12 patients were asymptomatic with heart murmurs occasionally found in routine physical examination.One patient underwent fistula ligation without cardiopulmonary bypass (CPB).The remaining 12 cases received fistula correction with beating heart CPB.Direct suture was used in 10 patients and autologous pericardial patch in other 2 patients.Two patients were associated with atrial septal defect (ASD) and underwent repair of ASD concurrently.The coronary artery aneurysm remained original shape without any intervention during the operation.The mean hospital delay was (11.0 ± 2.5) days.Two patients had decreased ejection fraction as low as 38％ within 3 days after the operation,but went up to over 50％ in follow-up 1 month later.Transient T wave change occurred in 7 patients,and another 2 patients showed a residual shunt with size of 1 to 2 millimeters through the fistula without further intervention after the surgical closure.All 13 patients had antiplatelet therapy with 12 taking Aspirin and one taking Dipyridamole.The dosage was 3-5 mg/(kg · d) with duration ranging from 3 days to 13 months with a median of 1 month.During the perioperative period and the follow-up period (3 months to 8 years),all patients were asymptomatically alive.Transthoracic echocardiography showed normal cardiac function.Compared with preoperative status,the diameter of dilated coronary arteries was not changed after the operation.There was no formation of thrombus in the coronary arteries.Electrocardiography showed no ST-T changes or arrhythmia or myocardial ischemia.Conclusions GCAA can be combined with congenital CAF in children,so it needs early operation.The evidence-based intervention of coronary artery aneurysm and usage of anticoagulant and antiplatelet therapy in pediatric patients was still lacking,which needs long-term follow-up.
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Objective Analyze the surgical and follow-up outcomes of congenital coronary artery fistula(CAF) in children and to discuss the treatment methods.Methods The clinical data were analyzed retrospectively in 22 patients who were diagnosed as congenital CAF and received surgical treatment between July 2008 and January 2017 in Guangzhou Women and Children's Medical Center.There were 14 boys and 8 girls.The median age was 17 months ranging from 14 days to 12 years old.The median weight was 8.8 kilograms ranging from 3.0 to 31.0 kilograms.Results Two patients had shortness of breath.Three had growth retardation with recurrent respiratory infection or tachypnea.The remaining 17 patients were asymptomatic.Twenty had heart murmurs.Fistulas originated from right coronary artery accounted for 11 patients,with 10 from left coronary artery,1 from both right and left coronary arteries.Fistulas drained into coronary sinus in 1 patient,right atrium in 6 patients,right ventricular in 13 patients and left ventricular in 2 patients.Single fistula occurred in 20 patients and multiple fistulas in 2 patients.The coronary arteries were obviously dilated in all patients with diameter from 4.0 to 12.0 millimeters.There were 8 cases with aneurysmal dilation of coronary arteries.Two patients underwent fistula ligation without cardiopulmonary bypass(CPB).The remaining 20 cases received fistula correction with beating heart CPB.Direct suture was used in 3 patients for closure of fistula inlet and 11 children for outlet.Both inlet and outlet were closed in 6 patients,and in 2 of them autologous pericardial patch was used for outlet.Other associated intra-cardiac anomalies in 7 children were corrected during the operation.The aneurysmal coronary arteries remained original shape without any intervention during the operation.The mean hospital delay was(12.3±3.2) days.Two patients appeared decreased heart function within 3 days after the operation,and recovered in return visit one month later.Another 2 patients showed a residual shunt with size of 1 to 2 millimeters through the fistula without further intervention after the surgical closure.During the perioperative period and the follow-up period(3 months to 8 years),all 22 patients were asymptomatically alive without regular anticoagulation management.Transthoracic echocardiography showed normal cardiac function.Compared with preoperative status,the diameter of dilated coronary arteries was reduced over 6 months follow up after the operation.There were no formation of thrombus in the coronary arteries.Electrocardiography showed no ST-T changes or arrhythmia or myocardial ischemia.Conclusion Clinical symptoms can be appeared in children with congenital CAF due to large shunt.Surgical correction is an effective method for the management of single CAF or CAF with coexisted intra-cardiac anomalies.The outcome was well.
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Objective To summarize the clinical characteristics,imaging characteristics,treatment and progno-sis of unilateral pulmonary vein atresia (UPVA)in children and to improve the clinician′ s understanding of this disease. Methods The clinical data of 4 cases of UPVA from January 2014 to December 2016 in Department of Cardiology,Guangzhou Women and Children′s Medical Center were retrospectively analyzed,and 50 cases from reviews of PubMed,OVID and Elsevier in the international medical literature database and 4 cases in Wanfang database for the domestic report were reviewed. The clinical characteristics,diagnosis,treatment and prognosis of total of 58 cases were analyzed. Results Four patients,with an average age of 1. 8 years (1. 5 - 2. 7 years),showing congenital UPVA in 3 patients and secondary UPVA in 1 patient. There was 1 case of left upper pulmonary vein atresia,2 cases of left pulmo-nary vein atresia and 1 case of right pulmonary vein atresia. Three cases showed recurrent hemoptysis and recurrent cough occurred in 1 case. Three cases were complicated with congenital heart disease. There was one case underwent bronchial artery embolization,1 case received pulmonary vein left atrium connection,and 2 cases received conservative treatment. All patients had been followed up for 1 - 3 years so far. The patient receiving pulmonary vein left atrium had been completely cured,and the patient receiving bronchial artery embolization showed no occurrence of hemoptysis but still showed recurrent cough hemoptysis. The patient with secondary UPVA had no obvious clinical symptoms,the other 1 case who rejected operation and received conservative treatment still had recurrent pulmonary infection,intermittent hemoptysis. The average age of 54 cases(40 cases with age ≤18 years old)from the literature reports was 13. 76 years (8 days - 43 years)in which 52 cases were diagnosed as congenital UPVA,while 2 cases were secondary UPVA. Twenty - seven cases were right pulmonary vein atresia,22 cases were left pulmonary vein atresia,and 5 cases were other types. There were 94. 4％(51 / 54 cases)of the patients having recurrent cough,pulmonary infection,92. 6％(50 /54 cases)of the patients with exertional dyspnea and polypnea,68. 5％(37 / 54 cases)of the patients with hemoptysis and hematemesis. There were 50. 0％(27 / 54 cases)of UPVA patients who were complicated with heart malformation. Different degrees of pulmonary hypertension were observed in 75. 9％(41 / 54 cases)of children,and 35. 2％(19 / 54 cases)of patients had pulmonary lymphatic dilatation. Pulmonary resection was performed in 25 cases,pulmonary vein left atrium connection was performed in 11 cases,bronchial artery embolization was performed in 7 cases,and conserva-tive treatment was performed in 11 cases. After operation,most of the patients had good prognosis without obvious clini-cal symptoms or mild symptoms. Conclusions In clinical practice,if unexplained hemoptysis,recurrent lower respira-tory tract infection,pulmonary consolidation,pulmonary dysplasia or pulmonary hypertension present,the possibility of UPVA should be considered. Early diagnosis and early bronchial artery embolization,reconstruction of the pulmonary vein and atrial connection and repair of the defect of heart,can improve the symptoms or cure the children and reduce the morta-lity significantly.
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Objective To summarize the clinical characteristics, follow-up and prognosis of Kawasaki disease (KD) in infants under 3 months. Method The clinical data of infants under 3 months diagnosed with KD during January 2009 to December 2013 were analyzed retrospectively. Results In a total of 1504 cases diagnosed with KD during during the study period, there were 40 (2.66%) infants under 3 months (30 males and 10 females). Except for fever, the frequencies of the other 5 main clinical manifestations were less than 50%. Laboratory tests suggested inflammatory reactions, thrombocytosis, anemia, and so on. Coronary artery disease was found in 32 cases (80%), including 24 cases of coronary artery dilatation, 8 cases of coronary artery aneurysm and 3 cases of coronary giant aneurysm. By the end of the follow-up period, there were no myocardial ischemia, myocardial infarction and death in all the patients; coronary artery diameter was normal in 37 cases (92.5%); 3 cases of giant coronary artery aneurysm still had coronary artery aneurysm and thrombosis. Conclusion The clinical manifestations of KD in little infants were atypical, the incidence of coronary artery disease is high, and color doppler echocardiography should be performed in time.
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Objective To summarize the clinical features,treatment and prognosis of infantile hepatic hemangioendothelioma(IHHE) combined with congestive heart failure(CHF) in children.Methods A retrospective analysis was made with the clinical data from 4 children with IHHE combined with CHF in Department of Pediatric Cardiology,Affiliated Guangzhou Women and Children's Medical Center of Guangzhou Medical University from May 2013 to December 2015.Results Among 4 infants,there were 3 male and 1 female,mean aged of 109 days (21-219 days),the average weight of 4 350 g (2 750-6 500 g),and 1 case was admitted because of abdominal swelling and respiratory distress,1 case of abdominal swelling,jaundice,shortness of breath admission,1 case of fever and shortness of breath admission,and 1 case of cyanosis and shortness of breath admission.Two patients were associated with hemangioma and Kasabach-Merritt syndrome.Three cases had multiple liver hemangioma scattered in the left lobe and right lobe of the liver,and 1 case had solitary liver hemangioma scattered in the right lobe.Tumor size diameters were ranged from 2-10 mm.Four children had been used Prednisone [1-2 mg/(kg · d)] and Digoxin,Dopamine,Furosemide,Spironolactone.Two cases responsed well to the therapy and were followed up for 2 years or 1 year and 6 months respectively,whose heart size,heart function and pulmonary hypertension became normal.One case with Kasabach-Merritt syndrome responsed poorly to the therapy.Two cases experienced transcatheter hepatic arteriovenous fistula embolization by using coil occlusion of hepatic artery-venous fistula.The postoperative hepatic tumor volume was significantly decreased,and the heart function was improved.One patient was followed up for 1 year and 6 months,and the heart size,pulmonary hypertension and heart function returned to normal.One case was still under follow-up.One case with Kasabach-Merritt syndrome died from heart failure and severe infection before surgery.The mortality rate was 25％ (1/4 cases).Conclusions IHHE is a rare cause of CHF in children.If heart failure is found unexplained in children,abdominal ultrasound or CT examination should be done immediatey in order to confirm the presence or absence of IHHE.The mortality rate in IHHE associated with CHF is very high.Early identification,early intervention,especially Prednisone combined with transcatheter hepatic arteriovenous fistula embolization can significantly improve heart function,reduce the mortality and improve the prognosis of IHHE associated with CHF in children.
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Objective Investigate the clinical features,diagnosis and treatment of the scimitar sydrome.Methods A retrospective analysis of clinical data of six children with scimitar syndrome from 2008 to 2014,including clinical symptoms and signs,chest X-ray,echocardiography,chest CT and cardiac catheterization,treatment outcome and follow-up and review of the literature.Results Six patients with scimitar syndrome were diagnosed between 2008 and 2014.There were 3 girls and 3 boys;5 cases ＜ 6 kg in weight.Scimitar syndrome was suspected in 5 cases because of extroversion,and diagnosed by color Doppler echocardiography in all 6 when a scimitar vein was detected entering the inferior vena cava.Computed tomography confirmed the diagnosis in all patients.One patient had horseshoe lung,3 had a unilobar right lung,3 had a hypoplastic right lower lobe.Three patients had severe pulmonary arterial hypertension,1 had moderate to severe pulmonary arterial hypertension,and 2 had moderate pulmonary arterial hypertension.All patients had lower respiratory tract infections,volume loss of the right lung,a normal or hyperinflated left lung,dextroversion of the heart,and scimitar arteries from the descending aorta.4 cases of pulmonary venous drainage correction surgery,3 underwent body-pulmonary embolism,1 case of 9-year-old asymptomatic children without surgery,and 1 patient with heart failure,severe pulmonary hypertension,pulmonary infection,septicemia died before surgery.Conclusion Clinically,found children with heart of dextrocardia position,growth retardation,recurrent lung infections,unexplained right heart failure,pulmonary hypertension,should consider the possibility scimitar syndrome.Early diagnosis and early treatment of pulmonary venous drainage and pulmonary vascular embolization,can significantly improve the clinical symptoms in children and reduce mortality.
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Objective To describe early clinical experience with the new amplatzer ductal occluder Ⅱ (ADO Ⅱ) for transcatheter patent ductus arteriosus(PDA) in children.Methods Twelve children were diagnosed as PDA from Jan.2013 to Apr.2014 in Guangzhou Children and Women's Hospital.All the children who were treated with the ADO Ⅱ had the indication of a successful interventional therapy successfully.The size of device was chosen according to aortographic and transthoracic echocardiography(TTE) results and pulmonary pressure.The device was delivered in a consequent or reverse way depending on the type of PDA,the minimal diameter of PDA and the size of duct ampulla.The device was delivered in a reverse way in ten patients,and two in a consequent way before detaching the device.Another aortogram was performed in order to check the position and form of the device,the velocities of blood flow in left pulmonary artery and the descending aorta though TTE and whether there was a residual shunt.All patients were examined by TTE in 24 hours after surgery and discharged without any complications 2 days later.The patients were programmed for the cardiologic consult including an TTE and electrocardiogram in 1,3,6 and 12 months after discharge.Results Twelve patients(7 male,5 female) with a median age of(1.59 ± 1.10) years(range 0.53-4.47 years),a median weight of (9.52 ± 3.41) kg(range 5.5-18.3 kg),a median pulmonary blood flow/systemic blood flow (Qp/ Qs) of 1.64 ± 0.45 (range 1.33-2.85),a median pulmonary artery systolic pressure (32.50 ± 10.05) mmHg (range 23-58 mmHg,1 mmHg =0.133 kPa),and the minimum (2.40 ±0.68) mm (1.6-3.8 mm),underwent transcatheter ductal closure with the ADO Ⅱ.Device sizes used were 3 mm ×4 mm(n =7),3 mm ×6 mm(n =3),6 mm ×6 mm (n =2),respectively and delivered with 4 or 5 F delivery catheters.The median fluoroscopy time was (6.39 ± 4.16) min(range 3.2-18.2 min).Complete ductal occlusion was achieved by the end of the procedure in 10 patients.The TTE showed good position of the occlusion and the velocities of blood flow in left pulmonary artery and the descending aorta were in a normal range.There was a trivial residual shunt after the surgery of 2 patients.No residual shunt was found after 24 hours in all 12 patients.In 1 case,the patient had a descending aortic obstruction with pressure gradient of 11 mmHg.Three months after surgery,the pressure descended to 10 mmHg by TTE.Complete ductal occlusion without aortic arch or left pulmonary artery stenosis had been identified in other 11 remaining patients on TTE follow-up of 6 months of 3 patients and 12 months of 6 patients.Conclusions The ADO Ⅱ achieves excellent ductal closure rates through low profile delivery systems in small infants and children with moderate and small PDA or morphologically varied PDAs.It is simple in use with few complications.Occlusion design allows closure with arterial or venous approach and delivery with 4 or 5 F delivery catheters.The children who used arterial approach,transthoracic echocardiography TTE is recommended to replace aortic angiography,so as to avoid puncturing the aorta and reduce vascular injury.