ABSTRACT
We report a case of extracardiac rupture of the left Valsalva sinus aneurysm, which is an extremely rare and fatal lesion. The three drugs, clindamycin, ethambutol and rifampicin, had been administered for eleven years because of lung mycobacterium infection. An emergency surgery was performed because of cardiac tamponade. The left Valsalva sinus was entirely enlarged without an aneurysmal neck. The other Valsalva sinuses seemed to be almost normal. The aneurysmal wall adhered the pulmonary artery and the left atrium. The left descending and circumflex arteries independently originated from the aneurysmal wall. The left main trunk seemed to become a part of the aneurysmal wall. Additionally the intraoperative transesophageal echocardiography showed severe aortic regurgitation. The Bio-Bentall procedure was performed. The right coronary artery was reconstructed with the Carrel patch method and the saphenous vein grafts were anastomosed to the proximal portions of the left anterior descending and circumflex arteries, individually. On the thirty-third postoperative day, the patient was discharged uneventfully expect for the delayed sternal closure on the second postoperative day. Five and half years after surgy, the patient is living a normal life and rifampicin has been administered without any anticoagulation drugs.
ABSTRACT
OBJECTIVE@#To investigate the morbidity of congenital heart defects(CHDs) in children with anorectal malformation, and to summarize appropriate treatment.@*METHODS@#The clinical data and echocardiographic findings of 155 children with congenital anorectal malformations from the Third Affiliated Hospital of Zhengzhou University during January 2016 and October 2019 were reviewed. According to the surgical findings of anorectal malformations, the patients were categorized as the high/intermediate group and the low group; the CHDs were classified as minor CHDs and major CHDs. Multiple logistic regression was used to analyze the correlation of wingspread classification, and extracardiac malformations with the severity of CHDs.@*RESULTS@#Out of 155 children with anorectal malformations, 47 (30.3%) had different types of cardiac structural malformations, including 18 cases of minor CHDs (11.6%) and 29 cases of major CHDs (18.7%). Sixty children (38.7%) had extracardiac malformations, of which 38 cases (24.5%) had a single extracardiac malformation, 15 cases (9.7%) had multiple extracardiac malformations, 6 had trisomy 21 syndrome, and 1 had VATER syndrome. Multivariate logistic regression analysis showed that wingspread classification of anorectal malformation and extracardiac disorders were independent risk factors for major CHDs. The probability of major CHDs in children with high/intermediate anorectal malformation was 4.709 times higher than that with low anorectal malformation (@*CONCLUSIONS@#The morbidity of major CHDs is higher in severe cases with high/intermediate anorectal malformation and acute cases without fistula or with obstructed fistula and cases with multiple congenital disorders. Echocardiography can define the type and severity of CHDs, which are useful to develop the optimal diagnosis and treatment plan for children with anorectal malformation.
Subject(s)
Child , Humans , Abnormalities, Multiple , Anorectal Malformations/therapy , Heart Defects, Congenital/mortality , Retrospective StudiesABSTRACT
@#Objective To investigate the surgery experience of modified intra/extracardiac conduit total cavopulmonary connection (TCPC). Methods We retrospectively analyzed clinical data of 47 patients of complex congenital heart disease undergoing intra/extracardiac conduit total cavopulmonary connection in our hospital between January 2008 and December 2015. There were 29 males and 18 females with a median age of 7 years (range 4 to 9 years) and median body weight of 22 kg (range 14 to 38 kg). The heart echocardiography and cardiac imaging confirmed diagnosis suitable for TCPC surgery. Results There was no early death in the whole group. The mean pulmonary arterial pressure was 16 (12–20) mm Hg and the ventilation time was 14 (7–97) h. The main complications were intractable pleural effusion in 7 patients, low cardiac output syndrome in 3 patients, repeated supraventricular tachycardia in 1 patient. All the patients recovered after treatment. At the end of discharge, the percutaneous oxygen saturation was 85%–96% (92.6%±3.3%). The echocardiography showed the conduit pressure was 0–2 mm Hg. Patients were followed up for 1 to 7 years. Three patients were lost. One patient had intestinal nutrition loss, receving repeated pleural effusion, the treatment was ineffective, died after 4 years. Four patients of repeated pleural effusion improved after treatment. One patient repeated attacks supraventricular tachycardia within 1 year, controlled by amiodaronum, already stopped about 28 months. No recurrence occurred. All survivors were in New York Heart Association (NYHA) functional class Ⅰ or Ⅱ, with good activity tolerance. Conclusion The modified intra/extracardiac conduit TCPC combines the advantages of both the lateral tunnel and the extracardiac conduit. The operation is simple, used in the treatment of complex congenital heart disease. The short-term and mid-term results are encouraging.
ABSTRACT
Objective: To quantitatively study the incidental extra-cardiac ifndings (ECFs) by coronary computed tomography angiography (CCTA) in patients with suspected coronary artery disease (CAD) in order to better recognize those lesions in clinical practice. Methods: A total of 1169 suspected CAD patients received CCTA in our hospital from 2011-06 to 2013-03 and 1030 patients were enrolled in this study. There were 589 in-patients, 441 out-patients and 549 patients≥60 years of age,481 patients Results: There were 197/1030 (19.1%) patients having 224 ECFs and 27 (2.6%) patients having 2 ECFs; 90/1030 (8.7%) patients having 106 signiifcant lesions including 3 (0.3%) of lung cancer and 8 (0.8%) of pulmonary embolism; 107 patients with 118 lesions without signiifcance. ECFs were found in 114/589 (19.4%) in-patients and in 83/441 (18.8%) out-patients, P>0.05; 76/481 (15.8%) of patients Conclusion: Unexpected ECFs detection rate was 19.1% in patients undergoing CCTA without further radiation exposure by reconstruction with the full FOV setting, and 8.7% of ECFs had clinical signiifcance. Radiologists should routinely analyze the extra-cardiac organs in CCTA.
ABSTRACT
Objective To sum the clinical experiences of the treatment in congenital heart disease single ventricle with extracardiac conduit (EC) fenestration.Methods Ninety-five patients diagnosed with univentricular heart disease underwent EC using Gore-Tax conduits at the Department of Children's Heart Center,Justus-Liebig-University Giessen Germany from June 1996 to July 2010.According to EC with or without fenestration,the patients were divided into two groups.Seventy-one routine fenestration of the extracardiac conduit (the fenestration group),58 patients (58/71) of children with high-risk preoperative intraoperative fenestration,13 cases (13/71) were due to low cardiac Rankinginterventional fenestration; extracardiac conduit fenestration (non-windowed group) did not undergo surgery in 24 patients (24/95).Results The fenestration had no death; Three died in non-windowed group.Postoperative effusions and postoperative mean pulmonary artery pressure in group without fenestration ((14.2 ± 2.3) d and (15.1 ± 3.4) mm Hg respectively) were significantly higher than group with fenestration ((10 ± 3.2) d and (13.2 ± 2.8) mm Hg respectively).It had significant differences(P =0.016).In the group with fenestration and without fenestration,postoperative oxygen saturation((90.3 ±4.0)％,(91.7 ±5.2)％),postoperative thrombosis (11.3％ (8/71),12.5％ (3/24)),and postoperative neurological problems (18.3％ (13/71),20.8％ (5/24)) did not differ between cohorts.Conclusion It is effective and safe to treat congenital heart disease single ventricle with EC.EC with fenestration can improve acute postoperative mortality by rising cardiac output,thereby can reduce early postoperative mortality.
ABSTRACT
Objective Multidector computed tomography (MDCT) is now commonly used for the evaluation of coronary artery disease. Because MDCT images include many non-cardiac organs and the patient population evaluated is highly susceptible to extracardiac diseases, this study was designed to evaluate the prevalence of extracardiac findings in the MDCT evaluation of ischemic heart disease. Methods From March 2007 to March 2008, a total of six-hundred twenty patients, who underwent 64-slice MDCT evaluations for chest pain, or dyspnea, were enrolled in this study. Cardiac and non-cardiac findings were comprehensively evaluated by a radiologist. Results Enrolled patients included 306 men (49.4%), with a mean age of 66 years. Significant coronary artery stenosis was found in 41.6%of the patients. A total of 158 extracardiac findings were observed in 110 (17.7%) patients. Commonly involved extracardiac organs were lung (36.7%), hepatobiliary system (21.5%), thyroid (19.6%), kidney (10.8%), spine (9.7%) and breast (0.6%). Of those 110 patients, 50 (45.5%) patients underwent further diagnostic investigations. Malignant disease was detected in three (2.7%) patients (lung cancer, pancreatic cancer, and thyroid cancer). Conclusions Extracardiac findings are frequently present and should be a concern in the MDCT evaluation of chest pain syndrome.
ABSTRACT
A sinus of Valsalva aneurysm is a rare cardiac disorder, and reports of it with an anomalous origin of the coronary artery are scarce. A 35-year-old male was admitted to our department with fatigue and cough. Multi-detector-row computer tomography (MDCT) revealed an isolated extracardiac right sinus of Valsalva aneurysm with an anomalous origin of the left circumflex artery (LCX) and total occlusion of the right coronary artery (RCA). Its diameter was about 70 mm. We performed a partial aortic root remodeling procedure with a trimmed J-graft because he had neither aortic regurgitation (AR) nor annuloaortic ectasia (AAE). Concomitantly, coronary artery bypass grafting to the RCA (Seg. 3) using a saphenous vein, and reconstruction of the LCX by Piehler's technique using a saphenous vein were added. The patient's postoperative course was uneventful, and he was discharged on the 28th postoperative day. Postoperative MDCT revealed that the aneurysm of the right sinus of Valsalva was not enhanced, and the RCA and LCX were patent. This procedure preserved the patient's own normal aortic valve and sinus of Valsalva and enables him to have more physiologically normal hemodynamics than aortic root reconstruction using a composite graft, e.g. Bentall procedure, Cabrol procedure, although the potential progression of the AR requires careful follow-up.
ABSTRACT
PURPOSE: Despite extracardiac conduit Fontan had many advantages, long-term results related to longevity of conduit, and anticoagulation were not proven. This study was to evaluate the long-term outcome of hospital survivors with extracardiac Fontan circulation. METHODS: Between 1996 and 2006, 200 patients underwent extracardiac conduit Fontan operation. Median age at the Fontan operation was 3.4 years (range:16 months-35.7 years). All patients (89.5 %) except 21 patients of one stage Fontan operation underwent bi-directional cavopulmonary shunt. Fenestration was required in 85 patients (42.5%). RESULTS: There has been 6 hospital mortalities (3.0%), and 7 late mortalities (3.6%) at a mean follow-up of 52.4+/-32.2 months (range; 18days-120 months). Overall 10-years survival was 92.4 2.1%. Multivariate analysis identified severe infection at early postoperative periods (hazard ratio =12.439, P=0.001), and high pulmonary arterial pressure at preoperative period (hazard ratio=3.445, P=0.038) as risk factors for mortality. Reoperation was performed in 24 patients (12.0%), and freedom from reoperation was 82.4+/-4.1% at 10 years. Arrhythmia occurred in 32 patients (16.0%) after Fontan operation, and freedom from arrhythmia was 85.14.4% at 10 years. Risk factors for arrhythmia were heterotaxy syndrome (P=0.001), Follow up duration (P=0.027) and the age at Fontan operation (P=0.001). Freedom from thromboembolism was 92.91.9% at 10 years. The conduit cross- sectional area decreased by 14%, and the extent of decrease of the conduit cross-sectional remained stable irrespective of the follow-up duration. 95.2% of patients had New York Heart Association class I. CONCLUSIONS: After 10 years of follow-up, the overall survival, and the functional status of survivors of the extracardiac Fontan procedure are satisfactory. Fenestration has a beneficial effect on the results of high-risk Fontan patients. The incidence of late deaths, reoperations, obstructions of the cavopulmonary pathway, arrhythmias and thromboembolisms is low.
Subject(s)
Humans , Arrhythmias, Cardiac , Arterial Pressure , Follow-Up Studies , Fontan Procedure , Freedom , Heart , Heart Bypass, Right , Heterotaxy Syndrome , Hospital Mortality , Incidence , Korea , Longevity , Mortality , Multivariate Analysis , Postoperative Period , Preoperative Period , Reoperation , Risk Factors , Survivors , ThromboembolismABSTRACT
BACKGROUND: This paper reviews our experience retrospectively to examine the clinical results and effectiveness of lateral tunnel (LT) and extracardiac conduit (ECC) Fontan procedures at a single institution. MATERIAL AND METHOD: One hundred and sixty five Fontan procedures were performed (67 LT and 98 ECC) between January 1996 and December 2006. Preoperative and postoperative hemodynamic values, arrhythmia, hospital and intensive care unit stay, chest tube drain, morbidity and mortality were reviewed. RESULT: The overall operative mortality in the LT and ECC groups was 4.5% (3) and 2.0% (2), respectively. There was a significant difference in the immediate postoperative transpulmonary gradient (LT 8.5+/-2.5 vs ECC 6.6+/-2.4, p-value<0.001) and central venous pressure (LT 18.3+/-3.8 vs ECC 15.6+/-2.4, p-value=0.001) between the two groups. The mean follow-up in the LT and ECC groups was 74.1+/-31.5 and 38.1+/-29.1 months, respectively. There was one late death. The actuarial survival at 10 years in the LT and ECC groups was 92% and 89%, respectively. In arrhythmia, the ECC patients showed a slightly low incidence but the difference was not statistically significant. CONCLUSION: Both the LT and ECC Fontan procedures showed comparable early and mid-term outcomes in terms of the surgical morbidity and mortality, postoperative hemodynamics, and mid-term survival. The ECC Fontan procedure reduces the risk of arrhythmia in the follow up period.
Subject(s)
Humans , Arrhythmias, Cardiac , Central Venous Pressure , Chest Tubes , Follow-Up Studies , Fontan Procedure , Hemodynamics , Incidence , Intensive Care Units , Mortality , Retrospective StudiesABSTRACT
Objective: To summarize the experience of clinical application of extracardiac valved tubes in complex congenital heart disease. Methods: From September 1979 to December 2001, 131 patients with complex congenital heart disease underwent surgical treatment by using extracardiac valved tubes, including 22 cases of aortic valved homografts, 73 cases of swine pulmonary valved tubes and 36 cases of extracardiac tubes with anti-calcified swine pericardium valve. Results: The overall mortality rate was 17.5%. The mortality rate has decreased to 10.7% in the late 10 years, and 4.5% in recent 5 years, respectively. Conclusion: It was suggested that aortic and pulmonary valved homograft preserved by liquid nitrogen is the first choice for extracardiac valved tubes. Extracardiac tubes with anti-calcified swine pericardium valve can also be used and they easily available. A better postoperative haemodynamic effect can be achieved by proper placement of the tubes, choice of suitable size and positioning in good geometrical figure.
ABSTRACT
BACKGROUND: Follow-up studies have shown that although outcomes have improved substantially over time,results of the Fontan operation and its modifications remain suboptimal.In this study,we reviewed our experience with the extracardiac conduit Fontan operation,with a focus early and midterm change of internal diameter of PTFE conduit. MATERIAL AND METHOD: Between April 1997 and July 2000 were reviewed.Twelve patients (M:6,F:6,mean age 42.04 +/- 12.43months,mean body weight 13.80 +/- 1.94kg)underwent extracardiac conduit Fontan operation with expanded PTFE graft.Mean cardiopulmonary bypass time was 109.7 +/- 26.99minute and mean operation time was 455 +/- 89.51minute. Intraoperative fenestration was performed in 10 patients.The aortic cross clamping was not performed in all patients. RESULT: There was no early deaths and no postoperative dysrhythmia. Postoperative protein losing enteropathy and prolonged pleural effusion occurred in 1(8.3%)and 4 patients(33.3%).Conduit patency was evaluated by magnetic resonance imaging studies.A 9.84 +/- 3.84%mean reduction in conduit internal diameter and there was no statistical correlation between the change of internal diameter of conduit and the postoperative duration after partial correlation analysis(r=0.019,p=0.955). CONCLUSION: These results demonstrate that the extracardiac conduit Fontan operation provies good early and midterm results and may reduce the prevalence of late arrhythmia.And there is no correlation between the change of internal diameter of conduit and the postoperative duration after extracardiac conduit Fontan operation with the expanded PTFE graft conduit.
Subject(s)
Humans , Body Weight , Cardiopulmonary Bypass , Constriction , Follow-Up Studies , Fontan Procedure , Magnetic Resonance Imaging , Pleural Effusion , Polytetrafluoroethylene , Prevalence , Protein-Losing Enteropathies , TransplantsABSTRACT
In a patient with single ventricle associated with complex systemic and/or pulmonary venous drainage, intraatrial Fontan procedure is sometimes technically difficult due to the complex spatial relationship between their orifices in the atrium. We report a case of the modified extracardiac conduit Fontan procedure in a patient with a single ventricle in which the inferior vena cava and the hepatic vein drained separately into the atrium and the intraatrial orifice of the hepatic vein was abut to the orifice of the left lower pulmonary vein.
Subject(s)
Humans , Drainage , Fontan Procedure , Heart , Hepatic Veins , Pulmonary Veins , Vena Cava, InferiorABSTRACT
PURPOSE: In order to find out how frequently and what kind of extra-cardiac anomalies (CAs) is associated with congenital heart disease (CHD) in general and in each CAs, autopsies with congenital cardiovascular malformations were studied. METHODS: Autopsy files of Seoul National University Children's Hospital from 1991 to 1995 were reviewed to find cases of CHDs. Other extra-CAs were tabulated from the records of autopsy files. The study population was comprised of 156 cases (86 still birth or product of terminated pregnancy; 66 livebirth; 4 unknowns-whether still birth or livebirth). RESULTS: The sex ratio was 1.14:1 males to females. In still birth cases, gestational age ranged from 16 to 42 weeeks and in live birth cases, age ranged from 1 day to 1 year 7 months (mostly neonates). The ventricular septal defect (VSD) was the most common CA, followed by coarctation of aorta, tetralogy of Follot in this order of frequency. No extra-CA was found in 55 cases, 1 extra-CA in 54 cases, 2 extra-CAs in 35 cases, 3 or more extra-CAs in 12 cases. VSD was commonly associated with Edward syndrome and holoprosencephaly, tetralogy of Fallot with holoprosencephaly, and coarctation with cystic hygroma. The most commonly involved organ system was the central nervous system followed by the digestive system, facial anomaly, and so on. Chromosomal anomaly associated with CHDs was 24 cases. Common individual anomalies were cystic hygroma, Edward syndrome, holoprosencephaly, and so on. CONCLUSION: As the fetus and infants with CHD have high incidence of associated extra-CAs, collaborative works involving dysmorphologist, geneticist, pathologist, surgeons, and pediatric cardiologist are needed. For further studies of CA and extra-CAs, a systematic database andprecise medical records and interdepartmental collaboration are necessary.