Anomalous origin of the left coronary artery (AOLCA) from the right sinus of Valsalva constitutes a rare congenital coronary artery anomaly. Patients with an anomalous left main coronary artery face a significantly hi...Anomalous origin of the left coronary artery (AOLCA) from the right sinus of Valsalva constitutes a rare congenital coronary artery anomaly. Patients with an anomalous left main coronary artery face a significantly higher risk of sudden cardiac death compared to those with an anomalous right coronary artery. The anomalous coronary artery traversing between the ascending aorta and the pulmonary artery markedly heightens the risk of myocardial ischemia, arrhythmia, and sudden death. Symptomatic patients often exhibit a longer intramural course of the coronary artery, which may necessitate earlier intervention or influence the choice of surgical repair method. Surgical intervention is advocated for patients with this anomaly, even in the absence of symptoms. For anomalous aortic origin of a coronary artery from the opposite sinus of Valsalva with an intramural course, coronary unroofing is the preferred revascularization procedure. This report presents a case of AOLCA originating from the right sinus of Valsalva, treated surgically using the unroofing technique for the aortic intramural segment of the anomalous coronary artery traversing between the great vessels. The unroofing technique is recommended for treating AOLCA with an extensive intramural course that does not involve the commissure.展开更多
We report the case of a ten-year-old boy who had often presented with respiratory distress since born. Chest X-ray showed an enlarged right ventricle. Echocardiography demonstrated characteristic features of Fallot's...We report the case of a ten-year-old boy who had often presented with respiratory distress since born. Chest X-ray showed an enlarged right ventricle. Echocardiography demonstrated characteristic features of Fallot's tetralogy and the left pulmonary artery could not be visualized. However, cardiac catheterization disclosed that the left pulmonary artery had an anomalous origin in the ascending aorta. The patient underwent total surgical correction of the defects. Nine days later, he was discharged. We present the results of a literature review of the incidence, physiopathological, clinical, diagnostic, and surgical characteristics of this rare disease.展开更多
A female patient aged 3 months and 10 days was admitted to the cardiology department because of symptoms of heart failure.According to the echocardiography results,the patient received a diagnosis of primary endocardi...A female patient aged 3 months and 10 days was admitted to the cardiology department because of symptoms of heart failure.According to the echocardiography results,the patient received a diagnosis of primary endocardial fi broelastosis and was treated withγ-globulin,prednisone,digoxin,and diuretics.Coronary computed tomographic angiography and coronary angiography were performed as there was no improvement after 2 months of treatment.Finally,the patient received a diagnosis of anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA).ALCAPA is a rare congenital heart defect that can cause severe heart failure during infancy,and is easily misdiagnosed clinically.In this report,we show the process of misdiagnosis of the case and consult the relevant literature,hoping to improve the understanding and early diagnosis of ALCAPA.展开更多
Anomalous origin of the right coronary artery is a rare congenital anomaly, but is associated with sudden death. Originating from the opposite sinus of Valsalva, an interarterial?course and an intramural course are es...Anomalous origin of the right coronary artery is a rare congenital anomaly, but is associated with sudden death. Originating from the opposite sinus of Valsalva, an interarterial?course and an intramural course are especially considered as the risk factor for fatal cardiac events. Surgical indication remains controversial because many patients are asymptomatic. A 52-year-old man with anomalous origin of the right coronary artery with an interarterial?course concomitant with the left main trunk disease was resuscitated from cardiopulmonary arrest. It was likely to be attributed to the left main trunk disease, but anatomical structure of the right coronary artery suggests its possible involvement. Prophylactic bypass grafting for the right coronary artery was performed using saphenous vein graft without ligating native vessel to prevent future cardiac events, as well as revascularization of the left main trunk disease. All grafts were patent in one-year follow-up coronary angiography. Any cardiac event has not occurred.展开更多
Objectives: We sought to determine the long-term outcomes and mortality-associated factors after systemic-topulmonaryartery shunt (SPS) in patients with heterotaxy syndrome. Methods: We retrospectively analyzed allpat...Objectives: We sought to determine the long-term outcomes and mortality-associated factors after systemic-topulmonaryartery shunt (SPS) in patients with heterotaxy syndrome. Methods: We retrospectively analyzed allpatients with a functional single ventricle and heterotaxy syndrome who underwent SPS at our center from January2001 to April 2022. Results: This study involved 84 patients. Restrictive pulmonary blood flow requiringearly modulation was presented in 34 (40%) patients. Compared with patients without restrictive pulmonaryblood flow (N = 50 [60%]), the postnatal survival of these 34 patients was significantly lower at 10 years (log rank:p = 0.04), but the statistical significance disappeared at 20 years (log rank: p = 0.18). Among 31 patients whounderwent SPS, 11 (35%) had extracardiac total anomalous pulmonary venous connection (TAPVC). The survivalrate after SPS was 80% at 10 years. Cox regression analysis showed that extracardiac TAPVC (hazard ratio6.44, 95% confidence interval 1.23–33.7, p = 0.03) and pulmonary venous obstruction (PVO) at TAPVC repair(hazard ratio 11.2, 95% confidence interval 2.13–58.5, p = 0.004) were significantly associated with death. In25 patients who underwent bidirectional cavopulmonary shunt (BCPS), surgical interventions on the pulmonaryartery (PA) were performed after SPS in 7 of 9 patients with PA coarctation, 3 of 4 with non-confluent PAs, and4 of 12 with normal PAs. At SPS, primary central PA plasty was performed in three patients with PA coarctationand 2 with non-confluent PAs. There was no significant difference in the PA index before BCPS between patientswith and without primary central PA plasty (p = 0.49). Among 20 patients who underwent total cavopulmonaryconnection (TCPC), adverse events occurred in 7 (35%) patients, including death in 1 (5%), intervention for pulmonaryarteriovenous malformation (PAVM) in 3 (15%), and surgical intervention for PVO in 3 (15%). TheB-type natriuretic peptide concentration was significantly higher in patients with than without adverse events(p = 0.02). The adverse event-free survival rate after TCPC was 69% at 10 years. Conclusion: ExtracardiacTAPVC and PVO at TAPVC repair were significantly associated with death after SPS in patients who had heterotaxysyndrome with a single ventricle. Surgical interventions on the PA were frequently required after SPS inpatients with PA coarctation or non-confluent PAs. Although satisfactory survival was achievable after TCPC,late-onset PAVM and PVO remain concerns.展开更多
Tetralogy of Fallot(TOF)with total anomalous pulmonary vein connections(TAPVC)is a rare type of complex congenital heart disease among all TOF cases.Co-presentation of major aortopulmonary collateral arteries(MAPCAs)c...Tetralogy of Fallot(TOF)with total anomalous pulmonary vein connections(TAPVC)is a rare type of complex congenital heart disease among all TOF cases.Co-presentation of major aortopulmonary collateral arteries(MAPCAs)compensates for the lack of central pulmonary bloodflow and decreases the severity of right-to-left shunting in TOF.We present a case of a 2-year-old child with complex diagnoses of TOF,TAPVC,a large secun-dum atrial septal defect(ASD),and intraoperatively identified MAPCAs.She underwent surgery to repair the TAPVC,valve-sparing reconstruction of the right ventricular outflow tract,interventricular defect closure,and the creation of patent foramen ovale(PFO).After the operation,hemodynamic instability happened along with sudden blood pressure drop,desaturation,and increased central venous pressure,which subsided after adminis-tering inhalational nitric oxide(NO).A postoperative pulmonary hypertension crisis was suggested when the patient experienced recurrent symptoms after the termination of NO.Echocardiographicfindings of a D-shaped left ventricle(LV),right-to-left PFO shunt and high tricuspid valve gradientfirmly established the diagnosis.It was subsequently managed with continuous NO inhalation and sildenafil,which rendered a satisfactory outcome.Repaired TOF and TAPVC could be at particular risk of developing pulmonary hypertension crisis,especially in the presence of MAPCAs due to possible remodeling of the pulmonary vasculature.Furthermore,a relatively non-compliant LV function and small left atrial size may exacerbate the risk of developing postcapillary pulmonary hypertension after TAPVC repair.A successful postoperative outcome calls for a meticulous preoperative analysis of the anatomical lesions,as well as careful monitoring.展开更多
Anomalous origin of coronary arteries may be encountered coincidentally in the presence of unrelated pathology or when these are affected directly. This may directly be responsible for affecting the procedure or outco...Anomalous origin of coronary arteries may be encountered coincidentally in the presence of unrelated pathology or when these are affected directly. This may directly be responsible for affecting the procedure or outcome. Various types of anomalies of origin,?as well as course of coronary arteries,?have been classified in the past. Here we report 3 cases of anomalous origin of coronary arteries in different scenarios. First case had anomalous coronary with bicuspid aortic valve with dilated ascending aorta for which Bentall’s procedure was done, while the second and third cases were anomalous coronaries with coronary artery disease for which coronary artery bypass grafting was done.展开更多
A 55-year-old female known case of essential hypertension and diabetes was brought to the Emergency Department in a drowsy state with cardiogenic shock. At presentation systolic BP was 70 mm Hg, pulse rate was 44/min ...A 55-year-old female known case of essential hypertension and diabetes was brought to the Emergency Department in a drowsy state with cardiogenic shock. At presentation systolic BP was 70 mm Hg, pulse rate was 44/min and random blood sugar was 239 mg/dl. Troponin I was 8.07. Electrocardiography showed junctional rhythm and bradycardia. Ejection Fraction (LVEF) was 15% - 20%. Coronary angiography was done which revealed single vessel disease in LAD with anomalous origin of RCA from midsegment of LAD. Patient was immediately taken up for Off</span></span><span style="white-space:normal;"><span style="font-family:"">-</span></span><span style="white-space:normal;"><span style="font-family:"">pump Coronary Artery Bypass Grafting (OPCAB). Right saphenous vein graft was harvested and anastomosed proximally to aorta and distally to LAD on beating heart using Medtronic Octopus heart stabilizer and coronary shunts. Wound closed in layers after achieving complete hemostasis. Patient was taken off ventilatory support on first post-operative day and discharged in a stable condition on fourth post-operative day. Post-operative Echocardiography showed LVEF increased from 15% - 20% at time of admission to 38% and 52% after 3</span></span><span style="white-space:normal;"><span style="font-family:""> </span></span><span style="white-space:normal;"><span style="font-family:"">months and 12 months of surgery respectively.展开更多
Objective Reviewing the experience in diagnosis and treatment of cases with aorta-pulmonary septal defect and aortic origin of right pulmonary artery complicated with interruption of aortic arch. Methods Clinical data...Objective Reviewing the experience in diagnosis and treatment of cases with aorta-pulmonary septal defect and aortic origin of right pulmonary artery complicated with interruption of aortic arch. Methods Clinical data from medical records for 5 patients (4 boys and 1 girl,age from 1 to 18 years on admission) who had been treated in Wuhan Asia Heart Hospital between 2005 and 2009展开更多
Anomalous origin of left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary anomaly. In this study, we present all the ALCAPA patients which were admitted at our institution during April ...Anomalous origin of left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary anomaly. In this study, we present all the ALCAPA patients which were admitted at our institution during April 2007-December 2010. Retrospective review of these patients regarding their clinical presentation and the use of diagnostic modalities will be presented in this series. There were total of five patients, three male and 2 female, with age range of 2 - 12 months. The most common symptoms at presentation were tachypnea (4/5) and poor feeding with irritability (3/5). Electrocardiogram was abnormal in 2/5 cases and chest X ray revealed cardiome-galy with pulmonary congestion in 4/5 patients. Echocardiogram showed mitral valve regurgitation in 5/5 cases (3 with moderate and 2 with mild to moderate), Left ventricular dilatation/dysfunction in 4/5 patients, echogenic left ventricular papillary muscles in 4/5 patients and prominent right coronary with strong suspecision of ALCAPA in 4/5 patients. Coronary angiography was performed in 4/5 cases to confirm the diagnosis. We conclude that by thorough clinical assessment along with ECG and CXR, the diagnosis of ALCAPA can be strongly suspected. Echocardiogram can almost always make the diagnosis of ALCAPA and coronary angiography can confirm the diagnosis in rare atypical cases.展开更多
Intralobar sequestration (ILS) is a complex lung lesion where part of a lower lobe lacks communication with the tracheobronchial tree and receives an aberrant arterial blood supply from the systemic circulation. That ...Intralobar sequestration (ILS) is a complex lung lesion where part of a lower lobe lacks communication with the tracheobronchial tree and receives an aberrant arterial blood supply from the systemic circulation. That systemic artery is usually large, originating from the thoracic aorta or the abdominal aorta and its upper branches. We describe a case of ILS where a large systemic artery coming up from below the diaphragm is formed by the convergence of many very small serpiginous arteries coming up from the area of the celiac trunk and common hepatic artery: a kind of “rete mirabile”. This peculiar morphology was not described before.展开更多
We show a brief report of two common arterial trunk cases(CAT)with different arrhythmias and discuss anatomy,clinical and diagnostic management.The burden of volume and pressure overload of this cardiac malformation m...We show a brief report of two common arterial trunk cases(CAT)with different arrhythmias and discuss anatomy,clinical and diagnostic management.The burden of volume and pressure overload of this cardiac malformation may predispose to different types of arrhythmia before and after surgical repair.Because of labile hemodynamic state in this group of patients,prompt diagnosis of any arrhythmia is mandatory as the devastating factor on prognosis.The first patient with a diagnosis of CAT Type II Collett and Edwards(CE)had a particular history with HIV seropositive mother assuming antiretroviral therapy during pregnancy,who presented hyperbilirubinemia and liver dysfunction at birth,and re-entry atrial tachycardia after repair.The second patient had CAT Type I CE with a partial anomalous venous connection of left superior pulmonary vein and uncommon type of atrial tachycardia with dual AV nodal physiology.展开更多
Background:Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare but potentially life-threatening congenital heart defect. A retrospective analysis was carried out to elucidate t...Background:Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare but potentially life-threatening congenital heart defect. A retrospective analysis was carried out to elucidate the surgical outcomes of ALCAPA in infants and children using follow-up echocardiography. Methods: From September 2008 to March 2017, 26 children diagnosed with ALCAPA underwent left coronary re-implantation. All surviving patients received echocardiography during follow-up. Results: The mortality rate after the operation was 11.5%. Before repair, twenty patients (76.9%) presented with left ventricular (LV) dysfunction. The mean Z-score of the preoperative LV end-diastolic diameter was 4.42 ± 2.09. Mitral regurgitation (MR) was present in all patients. Two patients (7.7%), both with mitral valve prolapse, underwent mitral valve repair at the time of ALCAPA repair. Two children required postoperative extracorporeal membrane oxygenation. LV function normalized at a median time of 5.3 months (range:0.5–36.0 months). The Z-score of the LV end-diastolic diameter decreased simultaneously. The degree of MR gradually decreased in all surviving patients.All patients had patency of the proximal left coronary artery confirmed by echocardiography at the most recent follow-up. Six patients (26.1%) showed supravalvar pulmonary stenosis and seven patients (30.4%) showed right pulmonary stenosis during follow-up. Conclusions: Coronary re-implantation was effective for rebuilding a dual coronary system in patients with ALCAPA and resulted in progressive improved LV function and reduced functional MR. Echocardiography was valuable for evaluating the outcomes. LV function, the degree of MR, and possible complications could be detected with follow-up echocardiography.展开更多
Anomalous origin of left coronary artery from the pulmonary artery (ALCAPA) is a rare congenitalcardiac malformation. Here we report a case of ALCAPA with atrial septal defect (ASD) initially visualized by transth...Anomalous origin of left coronary artery from the pulmonary artery (ALCAPA) is a rare congenitalcardiac malformation. Here we report a case of ALCAPA with atrial septal defect (ASD) initially visualized by transthoracic echocardiography, subsequently confirmed by the coronary angiography.展开更多
Background At present, creation of a dual coronary system with direct aortic implantation is the preferred management for anomalous origin of left coronary artery from the pulmonary artery (ALCAPA). However, controv...Background At present, creation of a dual coronary system with direct aortic implantation is the preferred management for anomalous origin of left coronary artery from the pulmonary artery (ALCAPA). However, controversy still exists over the initial management of mitral regurgitation (MR) at the time of ALCAPA repair. Methods Between March 2005 and March 2015, 52 consecutive patients (20 males and 32 females) underwent direct aortic implantation of ALCAPA. Median age at surgery was 10 months (range, 2 to 193). Mean weight was 9.7_+7.8 kg. In order to facilitate the analysis, description of MR was given a numeric grade: 0 = none, 1 = trivial, 2 = mild, 2.5 = mild-moderate, 3 = moderate, 3.5 = moderate-severe, and 4 = severe. Results The LV shortening fraction (LVFS) demonstrated significant improvement between presentation and discharge (32.1±10.1% vs. 28.6±9.6%, P = 0.023), and there was also significant improvement in the mean MR grade between presentation and discharge (2.40+1.16 vs. 2.91+ 1.19, P = 0.001). There were 4 hospital deaths (7.7%). The median follow-up was 21 months (range 1.5 to 111), 3 patients (5.8%) were lost to follow-up, 1 patient required reoperation for mitral valve replacement. Significant improvement was seen in LVFS between discharge and late follow-up (38.0±6.0% vs. 32.1±10.1%, P = 0.001), however, the degree of MR did not dem- onstrate further change (2.29_+1.20 vs. 2.40+1.16, P = 0.541). Conclusion Creation of a dual coronary system with direct aortic implantation of the left coronary artery results in complete recovery of left ventricular function. Concomitant mitral valve (MV) repair for ALCAPA patients with moderate-severe and severe MR is helpful to early MV function recovery.展开更多
Background Anomalous origin of the left coronary artery cardiac lesion resulting in myocardial ischemia even infarction, from the pulmonary artery is a rare congenital morphological impairment and dysfunction of left ...Background Anomalous origin of the left coronary artery cardiac lesion resulting in myocardial ischemia even infarction, from the pulmonary artery is a rare congenital morphological impairment and dysfunction of left ventricle, together with mitral regurgitation. Here we will introduce our experience in the surgical repair of this kind of congenital lesion and the retrospective analysis about the improvement of left ventricular dimension and mitral regurgitation in early postoperative term. Method From May 1998 to July 2012, 38 consecutive patients with anomalous coronary artery from the pulmonary artery underwent surgical correction (33 received left coronary artery re-implantation, 4 left coronary artery ligation or primary closure, 1 Takeuchi procedure, and 10 simultaneous mitral valve plasty). Left ventricular dimension, mitral regurgitation, and ejection fraction, were measured by color Doppler echocardiography preoperatively, and 1 month after discharge. Results Hospital survival was 94.7% (2 in-hospital deaths). Ten paptients with more than moderate mitral regurgitation received simultaneous mitral plasty, one of whom was converted to mechanical prosthetic valve replacement. Mitral valve annuloplasty was applied in 9 cases of coronary re-implantation correction, 3 of whom also received additional mitral leaflet cleft repair. Meanwhile 8 patients underwent other different concomitant operations. Echocardiographic results for the survivals 1 month after discharge showed that left ventricular end-diastolic, endsystolic dimension decreased from 40.05 ± 5.56 mm and 28.94 ± 6.21 mm to 33.07 ± 6.82 mr, (P 〈 0.01) and 23.04 ±5.87 mm (P 〈 0.01) respectively. The average mitral regurgitation grade was also reduced from 2.36 ± 1.08 to 1.64 ± 93 (P 〈 0.05) in the group. All survival patients improved clinically and NYHA functional class decreased significantly from 2.37 ± 1.08 to 2.10 ±0.54 (P 〈 0.05). Conclusions The surgical repair of anomalous origin of the left coronary artery from the pulmonary artery is safe and effective, and can get satisfactory dimensional and functional improvement of the left ventricle in early term. Although controversial, concomitant mitral valve plasty can be helpful for critical patients with severe mitral regurgitation.展开更多
Background Anomalous Origin of Coronary Artery (AOCA), a congenital heart disease with low incidence, can cause sudden cardiac death in the young. It' s surgical treatment is scarcely performed in clinical practice...Background Anomalous Origin of Coronary Artery (AOCA), a congenital heart disease with low incidence, can cause sudden cardiac death in the young. It' s surgical treatment is scarcely performed in clinical practice, thus the special nursing needed during the surgery for this rare disease is largely unrecognized. Method There were 7 adult patients who had suffered from AOCA received corrective operation in our hospital in the last 10 years. Preoperative preparation, nursing cooperation and emergency procedure were analyzed and summarized. Result The AOCA corrective surgery was all successfully completed on these 7 patients. The nursing key points were drawn from these patients. Conclusion Key points of nursing cooperation in AOCA correction surgery include: understanding the condition, mastering pathological anatomy preoperative, making strict application of gradient temperature control ,observing the change of the disease, and carrying out all kinds of emergency preparation during surgery. Meanwhile, careful check in all cases and literature data, participate in preoperative discussion, and skilled techniques are also important to the success of surgery.展开更多
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly It demonstrated the combined effects of the absence of a normal coronary flow with a coronary steal and th...Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly It demonstrated the combined effects of the absence of a normal coronary flow with a coronary steal and the profound ischemia that can produce left ventricular dysfunction and mitral regurgitation. We here introduce the postoperative management of patients with repair of anomalous ori artery from the pulmonary artery, with an emphasis on its outcome. Methods Reco gin rds of the left coronary of 31 patients with anomalous origin of the left coronary artery from the pulmonary artery receiving surgery from 1998 to 2010 were reviewed retrospectively, 10 of which were treated with the mitral valve surgically at the same time. The age of patients was 4 months to 16 years (m kilograms), all of which were diagno edian, sed of anomalous origin of the left coronary artery from the pulmonary by echocardiography and cardiac catheterization. After surgery, electrocardiogram, echocardiography, arterial blood pressure, transcutaneous oxygen saturation and central venous pressure were monitored. Common postoperative complications in our group were analysed. And preoperative and postoperative data including area of mitral regurgitation, left ventricular systolic diameter and left ventricular distolic diameter were obtained. Cardiopulmonary bypass time and mechanical ventilation time of postoperative patients with no pneumonia were compared with those with pneumonia. Binary logistic regression was applied for the analysis of the risk factors of postoperative pneumonia. Results Of 31 patients, 30 survived after surgery with early mortality of 3.23%. One patient died of severe low cardiac output syndrome. Mechanical ventilation time was 4 hours to 168 hours hours (mean, 39.68 ± 50.52 hours; median, 18 hours). ICU stay was 16 hours to 425 hours (mean, 111.65 ± 127.03 hours; median, 44 hours). In our group, common postoperative complications were myocardial ischemia(n = 12, 36.4%), infection(n = 11, 33.3%) including pneumonia (n = 10, 30.3%), postoperative tachyarrhythmia(n = 5, 15.2%), low postoperative cardiac output(n = 2, 6.1%), endocarditis(n = 1, 3.0%). Compared with preop-erative data, postoperative data including area of mitral regurgitation, left ventricular systolic diameter and left ventricular distolic diameter decreased remarkably(P 〈 0.01), with left ventricular ejection fraction significantly improved (P 〈 0.05). Compared with mechanical ventilation time of patients with no postoperative pneumonia, time of those with pneumonia apparently prolonged(P 〈 0.01 ) while cardiopulmonary bypass time extended (P 〈 0.05). Mechanical ventilation time was a risk factor of postoperative pneumonia (OR = 1.041, OR 95%CI = (1.010, 1.073). Conclusions Proper therapy strategies according to the knowledge of the ALCAPA disease and the change of postoperative pathology and physiology play an important role in avoiding the postoperative complications and improving postoperative outcomes.展开更多
目的:分析成人冠状动脉异常主动脉起源(anomalous aortic origin of coronary artery,AAOCA)的病例特点,总结经胸超声心动图(transthoracic echocardiography,TTE)漏诊原因,为提高TTE诊断成人AAOCA的准确性提供参考。方法:选择2019年1月...目的:分析成人冠状动脉异常主动脉起源(anomalous aortic origin of coronary artery,AAOCA)的病例特点,总结经胸超声心动图(transthoracic echocardiography,TTE)漏诊原因,为提高TTE诊断成人AAOCA的准确性提供参考。方法:选择2019年1月至2022年12月在某院经冠状动脉CT血管造影和/或有创冠状动脉造影确诊AAOCA的患者37例,统计患者临床症状、12导联心电图、心肌酶检查及TTE检查结果;将患者按照冠状动脉异常起源部位进行分型,分析TTE漏诊原因。采用卡方检验比较TTE漏诊差异情况。结果:37例患者中,无症状或轻症者共31例;12导联心电图检查、心肌酶检查、心腔大小变化、节段性室壁运动异常及左室收缩功能结果均以阴性者居多。以右冠状动脉起源于左冠状动脉窦(anomalous origin of the right coronary artery from left sinus,ARCA-L)患者最多,占比为59.45%(22/37);TTE 2次检查共确诊21例患者,均为冠状动脉异常起源于对侧冠状动脉窦(anomalous origin of coronary artery arising from the opposite sinus,ACAOS),其中ARCA-L 19例,TTE对ACAOS的检出率达87.5%;TTE检查13例起源于分支、高位开口的患者全部漏诊。TTE对ACAOS的检出率要显著高于冠状动脉异常起源于分支和起源于高位开口的检出率,差异有统计学意义(21/24 vs 0/13,P<0.001)。结论:成人AAOCA患者症状表现及相关检查结果以阴性居多、缺乏特异性;TTE对ACAOS的诊断检出率较高,但对冠状动脉异常起源于分支、高位开口的患者易漏诊。超声医师需进行多切面多角度联合扫查,正确应用彩色多普勒血流显像甄别假阴性,以降低漏诊率。展开更多
文摘Anomalous origin of the left coronary artery (AOLCA) from the right sinus of Valsalva constitutes a rare congenital coronary artery anomaly. Patients with an anomalous left main coronary artery face a significantly higher risk of sudden cardiac death compared to those with an anomalous right coronary artery. The anomalous coronary artery traversing between the ascending aorta and the pulmonary artery markedly heightens the risk of myocardial ischemia, arrhythmia, and sudden death. Symptomatic patients often exhibit a longer intramural course of the coronary artery, which may necessitate earlier intervention or influence the choice of surgical repair method. Surgical intervention is advocated for patients with this anomaly, even in the absence of symptoms. For anomalous aortic origin of a coronary artery from the opposite sinus of Valsalva with an intramural course, coronary unroofing is the preferred revascularization procedure. This report presents a case of AOLCA originating from the right sinus of Valsalva, treated surgically using the unroofing technique for the aortic intramural segment of the anomalous coronary artery traversing between the great vessels. The unroofing technique is recommended for treating AOLCA with an extensive intramural course that does not involve the commissure.
文摘We report the case of a ten-year-old boy who had often presented with respiratory distress since born. Chest X-ray showed an enlarged right ventricle. Echocardiography demonstrated characteristic features of Fallot's tetralogy and the left pulmonary artery could not be visualized. However, cardiac catheterization disclosed that the left pulmonary artery had an anomalous origin in the ascending aorta. The patient underwent total surgical correction of the defects. Nine days later, he was discharged. We present the results of a literature review of the incidence, physiopathological, clinical, diagnostic, and surgical characteristics of this rare disease.
基金The project was funded by the Natural Science Foundation of Chongqing Science and Technology Commission(cstc2019jcyj-msxmX0866).
文摘A female patient aged 3 months and 10 days was admitted to the cardiology department because of symptoms of heart failure.According to the echocardiography results,the patient received a diagnosis of primary endocardial fi broelastosis and was treated withγ-globulin,prednisone,digoxin,and diuretics.Coronary computed tomographic angiography and coronary angiography were performed as there was no improvement after 2 months of treatment.Finally,the patient received a diagnosis of anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA).ALCAPA is a rare congenital heart defect that can cause severe heart failure during infancy,and is easily misdiagnosed clinically.In this report,we show the process of misdiagnosis of the case and consult the relevant literature,hoping to improve the understanding and early diagnosis of ALCAPA.
文摘Anomalous origin of the right coronary artery is a rare congenital anomaly, but is associated with sudden death. Originating from the opposite sinus of Valsalva, an interarterial?course and an intramural course are especially considered as the risk factor for fatal cardiac events. Surgical indication remains controversial because many patients are asymptomatic. A 52-year-old man with anomalous origin of the right coronary artery with an interarterial?course concomitant with the left main trunk disease was resuscitated from cardiopulmonary arrest. It was likely to be attributed to the left main trunk disease, but anatomical structure of the right coronary artery suggests its possible involvement. Prophylactic bypass grafting for the right coronary artery was performed using saphenous vein graft without ligating native vessel to prevent future cardiac events, as well as revascularization of the left main trunk disease. All grafts were patent in one-year follow-up coronary angiography. Any cardiac event has not occurred.
文摘Objectives: We sought to determine the long-term outcomes and mortality-associated factors after systemic-topulmonaryartery shunt (SPS) in patients with heterotaxy syndrome. Methods: We retrospectively analyzed allpatients with a functional single ventricle and heterotaxy syndrome who underwent SPS at our center from January2001 to April 2022. Results: This study involved 84 patients. Restrictive pulmonary blood flow requiringearly modulation was presented in 34 (40%) patients. Compared with patients without restrictive pulmonaryblood flow (N = 50 [60%]), the postnatal survival of these 34 patients was significantly lower at 10 years (log rank:p = 0.04), but the statistical significance disappeared at 20 years (log rank: p = 0.18). Among 31 patients whounderwent SPS, 11 (35%) had extracardiac total anomalous pulmonary venous connection (TAPVC). The survivalrate after SPS was 80% at 10 years. Cox regression analysis showed that extracardiac TAPVC (hazard ratio6.44, 95% confidence interval 1.23–33.7, p = 0.03) and pulmonary venous obstruction (PVO) at TAPVC repair(hazard ratio 11.2, 95% confidence interval 2.13–58.5, p = 0.004) were significantly associated with death. In25 patients who underwent bidirectional cavopulmonary shunt (BCPS), surgical interventions on the pulmonaryartery (PA) were performed after SPS in 7 of 9 patients with PA coarctation, 3 of 4 with non-confluent PAs, and4 of 12 with normal PAs. At SPS, primary central PA plasty was performed in three patients with PA coarctationand 2 with non-confluent PAs. There was no significant difference in the PA index before BCPS between patientswith and without primary central PA plasty (p = 0.49). Among 20 patients who underwent total cavopulmonaryconnection (TCPC), adverse events occurred in 7 (35%) patients, including death in 1 (5%), intervention for pulmonaryarteriovenous malformation (PAVM) in 3 (15%), and surgical intervention for PVO in 3 (15%). TheB-type natriuretic peptide concentration was significantly higher in patients with than without adverse events(p = 0.02). The adverse event-free survival rate after TCPC was 69% at 10 years. Conclusion: ExtracardiacTAPVC and PVO at TAPVC repair were significantly associated with death after SPS in patients who had heterotaxysyndrome with a single ventricle. Surgical interventions on the PA were frequently required after SPS inpatients with PA coarctation or non-confluent PAs. Although satisfactory survival was achievable after TCPC,late-onset PAVM and PVO remain concerns.
基金The report was conducted in accordance with the Nuremberg Code and Declaration of Helsinki,and the protocol was approved by the Institutional Review Board of National Cardiovascular Center Harapan Kita(No.LB.02.01/VII/037/KEP037/2022).
文摘Tetralogy of Fallot(TOF)with total anomalous pulmonary vein connections(TAPVC)is a rare type of complex congenital heart disease among all TOF cases.Co-presentation of major aortopulmonary collateral arteries(MAPCAs)compensates for the lack of central pulmonary bloodflow and decreases the severity of right-to-left shunting in TOF.We present a case of a 2-year-old child with complex diagnoses of TOF,TAPVC,a large secun-dum atrial septal defect(ASD),and intraoperatively identified MAPCAs.She underwent surgery to repair the TAPVC,valve-sparing reconstruction of the right ventricular outflow tract,interventricular defect closure,and the creation of patent foramen ovale(PFO).After the operation,hemodynamic instability happened along with sudden blood pressure drop,desaturation,and increased central venous pressure,which subsided after adminis-tering inhalational nitric oxide(NO).A postoperative pulmonary hypertension crisis was suggested when the patient experienced recurrent symptoms after the termination of NO.Echocardiographicfindings of a D-shaped left ventricle(LV),right-to-left PFO shunt and high tricuspid valve gradientfirmly established the diagnosis.It was subsequently managed with continuous NO inhalation and sildenafil,which rendered a satisfactory outcome.Repaired TOF and TAPVC could be at particular risk of developing pulmonary hypertension crisis,especially in the presence of MAPCAs due to possible remodeling of the pulmonary vasculature.Furthermore,a relatively non-compliant LV function and small left atrial size may exacerbate the risk of developing postcapillary pulmonary hypertension after TAPVC repair.A successful postoperative outcome calls for a meticulous preoperative analysis of the anatomical lesions,as well as careful monitoring.
文摘Anomalous origin of coronary arteries may be encountered coincidentally in the presence of unrelated pathology or when these are affected directly. This may directly be responsible for affecting the procedure or outcome. Various types of anomalies of origin,?as well as course of coronary arteries,?have been classified in the past. Here we report 3 cases of anomalous origin of coronary arteries in different scenarios. First case had anomalous coronary with bicuspid aortic valve with dilated ascending aorta for which Bentall’s procedure was done, while the second and third cases were anomalous coronaries with coronary artery disease for which coronary artery bypass grafting was done.
文摘A 55-year-old female known case of essential hypertension and diabetes was brought to the Emergency Department in a drowsy state with cardiogenic shock. At presentation systolic BP was 70 mm Hg, pulse rate was 44/min and random blood sugar was 239 mg/dl. Troponin I was 8.07. Electrocardiography showed junctional rhythm and bradycardia. Ejection Fraction (LVEF) was 15% - 20%. Coronary angiography was done which revealed single vessel disease in LAD with anomalous origin of RCA from midsegment of LAD. Patient was immediately taken up for Off</span></span><span style="white-space:normal;"><span style="font-family:"">-</span></span><span style="white-space:normal;"><span style="font-family:"">pump Coronary Artery Bypass Grafting (OPCAB). Right saphenous vein graft was harvested and anastomosed proximally to aorta and distally to LAD on beating heart using Medtronic Octopus heart stabilizer and coronary shunts. Wound closed in layers after achieving complete hemostasis. Patient was taken off ventilatory support on first post-operative day and discharged in a stable condition on fourth post-operative day. Post-operative Echocardiography showed LVEF increased from 15% - 20% at time of admission to 38% and 52% after 3</span></span><span style="white-space:normal;"><span style="font-family:""> </span></span><span style="white-space:normal;"><span style="font-family:"">months and 12 months of surgery respectively.
文摘Objective Reviewing the experience in diagnosis and treatment of cases with aorta-pulmonary septal defect and aortic origin of right pulmonary artery complicated with interruption of aortic arch. Methods Clinical data from medical records for 5 patients (4 boys and 1 girl,age from 1 to 18 years on admission) who had been treated in Wuhan Asia Heart Hospital between 2005 and 2009
文摘Anomalous origin of left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary anomaly. In this study, we present all the ALCAPA patients which were admitted at our institution during April 2007-December 2010. Retrospective review of these patients regarding their clinical presentation and the use of diagnostic modalities will be presented in this series. There were total of five patients, three male and 2 female, with age range of 2 - 12 months. The most common symptoms at presentation were tachypnea (4/5) and poor feeding with irritability (3/5). Electrocardiogram was abnormal in 2/5 cases and chest X ray revealed cardiome-galy with pulmonary congestion in 4/5 patients. Echocardiogram showed mitral valve regurgitation in 5/5 cases (3 with moderate and 2 with mild to moderate), Left ventricular dilatation/dysfunction in 4/5 patients, echogenic left ventricular papillary muscles in 4/5 patients and prominent right coronary with strong suspecision of ALCAPA in 4/5 patients. Coronary angiography was performed in 4/5 cases to confirm the diagnosis. We conclude that by thorough clinical assessment along with ECG and CXR, the diagnosis of ALCAPA can be strongly suspected. Echocardiogram can almost always make the diagnosis of ALCAPA and coronary angiography can confirm the diagnosis in rare atypical cases.
文摘Intralobar sequestration (ILS) is a complex lung lesion where part of a lower lobe lacks communication with the tracheobronchial tree and receives an aberrant arterial blood supply from the systemic circulation. That systemic artery is usually large, originating from the thoracic aorta or the abdominal aorta and its upper branches. We describe a case of ILS where a large systemic artery coming up from below the diaphragm is formed by the convergence of many very small serpiginous arteries coming up from the area of the celiac trunk and common hepatic artery: a kind of “rete mirabile”. This peculiar morphology was not described before.
文摘We show a brief report of two common arterial trunk cases(CAT)with different arrhythmias and discuss anatomy,clinical and diagnostic management.The burden of volume and pressure overload of this cardiac malformation may predispose to different types of arrhythmia before and after surgical repair.Because of labile hemodynamic state in this group of patients,prompt diagnosis of any arrhythmia is mandatory as the devastating factor on prognosis.The first patient with a diagnosis of CAT Type II Collett and Edwards(CE)had a particular history with HIV seropositive mother assuming antiretroviral therapy during pregnancy,who presented hyperbilirubinemia and liver dysfunction at birth,and re-entry atrial tachycardia after repair.The second patient had CAT Type I CE with a partial anomalous venous connection of left superior pulmonary vein and uncommon type of atrial tachycardia with dual AV nodal physiology.
文摘Background:Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare but potentially life-threatening congenital heart defect. A retrospective analysis was carried out to elucidate the surgical outcomes of ALCAPA in infants and children using follow-up echocardiography. Methods: From September 2008 to March 2017, 26 children diagnosed with ALCAPA underwent left coronary re-implantation. All surviving patients received echocardiography during follow-up. Results: The mortality rate after the operation was 11.5%. Before repair, twenty patients (76.9%) presented with left ventricular (LV) dysfunction. The mean Z-score of the preoperative LV end-diastolic diameter was 4.42 ± 2.09. Mitral regurgitation (MR) was present in all patients. Two patients (7.7%), both with mitral valve prolapse, underwent mitral valve repair at the time of ALCAPA repair. Two children required postoperative extracorporeal membrane oxygenation. LV function normalized at a median time of 5.3 months (range:0.5–36.0 months). The Z-score of the LV end-diastolic diameter decreased simultaneously. The degree of MR gradually decreased in all surviving patients.All patients had patency of the proximal left coronary artery confirmed by echocardiography at the most recent follow-up. Six patients (26.1%) showed supravalvar pulmonary stenosis and seven patients (30.4%) showed right pulmonary stenosis during follow-up. Conclusions: Coronary re-implantation was effective for rebuilding a dual coronary system in patients with ALCAPA and resulted in progressive improved LV function and reduced functional MR. Echocardiography was valuable for evaluating the outcomes. LV function, the degree of MR, and possible complications could be detected with follow-up echocardiography.
文摘Anomalous origin of left coronary artery from the pulmonary artery (ALCAPA) is a rare congenitalcardiac malformation. Here we report a case of ALCAPA with atrial septal defect (ASD) initially visualized by transthoracic echocardiography, subsequently confirmed by the coronary angiography.
基金supported by Guangdong Province Medical Scientific Research Foundation(No.A2016116)
文摘Background At present, creation of a dual coronary system with direct aortic implantation is the preferred management for anomalous origin of left coronary artery from the pulmonary artery (ALCAPA). However, controversy still exists over the initial management of mitral regurgitation (MR) at the time of ALCAPA repair. Methods Between March 2005 and March 2015, 52 consecutive patients (20 males and 32 females) underwent direct aortic implantation of ALCAPA. Median age at surgery was 10 months (range, 2 to 193). Mean weight was 9.7_+7.8 kg. In order to facilitate the analysis, description of MR was given a numeric grade: 0 = none, 1 = trivial, 2 = mild, 2.5 = mild-moderate, 3 = moderate, 3.5 = moderate-severe, and 4 = severe. Results The LV shortening fraction (LVFS) demonstrated significant improvement between presentation and discharge (32.1±10.1% vs. 28.6±9.6%, P = 0.023), and there was also significant improvement in the mean MR grade between presentation and discharge (2.40+1.16 vs. 2.91+ 1.19, P = 0.001). There were 4 hospital deaths (7.7%). The median follow-up was 21 months (range 1.5 to 111), 3 patients (5.8%) were lost to follow-up, 1 patient required reoperation for mitral valve replacement. Significant improvement was seen in LVFS between discharge and late follow-up (38.0±6.0% vs. 32.1±10.1%, P = 0.001), however, the degree of MR did not dem- onstrate further change (2.29_+1.20 vs. 2.40+1.16, P = 0.541). Conclusion Creation of a dual coronary system with direct aortic implantation of the left coronary artery results in complete recovery of left ventricular function. Concomitant mitral valve (MV) repair for ALCAPA patients with moderate-severe and severe MR is helpful to early MV function recovery.
基金supported by The Twelfth National Five-Year Plan (Grant No. 2011BAI11B22)
文摘Background Anomalous origin of the left coronary artery cardiac lesion resulting in myocardial ischemia even infarction, from the pulmonary artery is a rare congenital morphological impairment and dysfunction of left ventricle, together with mitral regurgitation. Here we will introduce our experience in the surgical repair of this kind of congenital lesion and the retrospective analysis about the improvement of left ventricular dimension and mitral regurgitation in early postoperative term. Method From May 1998 to July 2012, 38 consecutive patients with anomalous coronary artery from the pulmonary artery underwent surgical correction (33 received left coronary artery re-implantation, 4 left coronary artery ligation or primary closure, 1 Takeuchi procedure, and 10 simultaneous mitral valve plasty). Left ventricular dimension, mitral regurgitation, and ejection fraction, were measured by color Doppler echocardiography preoperatively, and 1 month after discharge. Results Hospital survival was 94.7% (2 in-hospital deaths). Ten paptients with more than moderate mitral regurgitation received simultaneous mitral plasty, one of whom was converted to mechanical prosthetic valve replacement. Mitral valve annuloplasty was applied in 9 cases of coronary re-implantation correction, 3 of whom also received additional mitral leaflet cleft repair. Meanwhile 8 patients underwent other different concomitant operations. Echocardiographic results for the survivals 1 month after discharge showed that left ventricular end-diastolic, endsystolic dimension decreased from 40.05 ± 5.56 mm and 28.94 ± 6.21 mm to 33.07 ± 6.82 mr, (P 〈 0.01) and 23.04 ±5.87 mm (P 〈 0.01) respectively. The average mitral regurgitation grade was also reduced from 2.36 ± 1.08 to 1.64 ± 93 (P 〈 0.05) in the group. All survival patients improved clinically and NYHA functional class decreased significantly from 2.37 ± 1.08 to 2.10 ±0.54 (P 〈 0.05). Conclusions The surgical repair of anomalous origin of the left coronary artery from the pulmonary artery is safe and effective, and can get satisfactory dimensional and functional improvement of the left ventricle in early term. Although controversial, concomitant mitral valve plasty can be helpful for critical patients with severe mitral regurgitation.
基金supported by The National Five-Year Plan(No.2011BAI11B22)Guangdong province science and technology plan(No.2011A030400001)
文摘Background Anomalous Origin of Coronary Artery (AOCA), a congenital heart disease with low incidence, can cause sudden cardiac death in the young. It' s surgical treatment is scarcely performed in clinical practice, thus the special nursing needed during the surgery for this rare disease is largely unrecognized. Method There were 7 adult patients who had suffered from AOCA received corrective operation in our hospital in the last 10 years. Preoperative preparation, nursing cooperation and emergency procedure were analyzed and summarized. Result The AOCA corrective surgery was all successfully completed on these 7 patients. The nursing key points were drawn from these patients. Conclusion Key points of nursing cooperation in AOCA correction surgery include: understanding the condition, mastering pathological anatomy preoperative, making strict application of gradient temperature control ,observing the change of the disease, and carrying out all kinds of emergency preparation during surgery. Meanwhile, careful check in all cases and literature data, participate in preoperative discussion, and skilled techniques are also important to the success of surgery.
基金supported by the National Science and Technology Infrastructure Program of the twelfth five-yearplan period(No:2011BAI11B22)
文摘Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly It demonstrated the combined effects of the absence of a normal coronary flow with a coronary steal and the profound ischemia that can produce left ventricular dysfunction and mitral regurgitation. We here introduce the postoperative management of patients with repair of anomalous ori artery from the pulmonary artery, with an emphasis on its outcome. Methods Reco gin rds of the left coronary of 31 patients with anomalous origin of the left coronary artery from the pulmonary artery receiving surgery from 1998 to 2010 were reviewed retrospectively, 10 of which were treated with the mitral valve surgically at the same time. The age of patients was 4 months to 16 years (m kilograms), all of which were diagno edian, sed of anomalous origin of the left coronary artery from the pulmonary by echocardiography and cardiac catheterization. After surgery, electrocardiogram, echocardiography, arterial blood pressure, transcutaneous oxygen saturation and central venous pressure were monitored. Common postoperative complications in our group were analysed. And preoperative and postoperative data including area of mitral regurgitation, left ventricular systolic diameter and left ventricular distolic diameter were obtained. Cardiopulmonary bypass time and mechanical ventilation time of postoperative patients with no pneumonia were compared with those with pneumonia. Binary logistic regression was applied for the analysis of the risk factors of postoperative pneumonia. Results Of 31 patients, 30 survived after surgery with early mortality of 3.23%. One patient died of severe low cardiac output syndrome. Mechanical ventilation time was 4 hours to 168 hours hours (mean, 39.68 ± 50.52 hours; median, 18 hours). ICU stay was 16 hours to 425 hours (mean, 111.65 ± 127.03 hours; median, 44 hours). In our group, common postoperative complications were myocardial ischemia(n = 12, 36.4%), infection(n = 11, 33.3%) including pneumonia (n = 10, 30.3%), postoperative tachyarrhythmia(n = 5, 15.2%), low postoperative cardiac output(n = 2, 6.1%), endocarditis(n = 1, 3.0%). Compared with preop-erative data, postoperative data including area of mitral regurgitation, left ventricular systolic diameter and left ventricular distolic diameter decreased remarkably(P 〈 0.01), with left ventricular ejection fraction significantly improved (P 〈 0.05). Compared with mechanical ventilation time of patients with no postoperative pneumonia, time of those with pneumonia apparently prolonged(P 〈 0.01 ) while cardiopulmonary bypass time extended (P 〈 0.05). Mechanical ventilation time was a risk factor of postoperative pneumonia (OR = 1.041, OR 95%CI = (1.010, 1.073). Conclusions Proper therapy strategies according to the knowledge of the ALCAPA disease and the change of postoperative pathology and physiology play an important role in avoiding the postoperative complications and improving postoperative outcomes.
文摘目的:分析成人冠状动脉异常主动脉起源(anomalous aortic origin of coronary artery,AAOCA)的病例特点,总结经胸超声心动图(transthoracic echocardiography,TTE)漏诊原因,为提高TTE诊断成人AAOCA的准确性提供参考。方法:选择2019年1月至2022年12月在某院经冠状动脉CT血管造影和/或有创冠状动脉造影确诊AAOCA的患者37例,统计患者临床症状、12导联心电图、心肌酶检查及TTE检查结果;将患者按照冠状动脉异常起源部位进行分型,分析TTE漏诊原因。采用卡方检验比较TTE漏诊差异情况。结果:37例患者中,无症状或轻症者共31例;12导联心电图检查、心肌酶检查、心腔大小变化、节段性室壁运动异常及左室收缩功能结果均以阴性者居多。以右冠状动脉起源于左冠状动脉窦(anomalous origin of the right coronary artery from left sinus,ARCA-L)患者最多,占比为59.45%(22/37);TTE 2次检查共确诊21例患者,均为冠状动脉异常起源于对侧冠状动脉窦(anomalous origin of coronary artery arising from the opposite sinus,ACAOS),其中ARCA-L 19例,TTE对ACAOS的检出率达87.5%;TTE检查13例起源于分支、高位开口的患者全部漏诊。TTE对ACAOS的检出率要显著高于冠状动脉异常起源于分支和起源于高位开口的检出率,差异有统计学意义(21/24 vs 0/13,P<0.001)。结论:成人AAOCA患者症状表现及相关检查结果以阴性居多、缺乏特异性;TTE对ACAOS的诊断检出率较高,但对冠状动脉异常起源于分支、高位开口的患者易漏诊。超声医师需进行多切面多角度联合扫查,正确应用彩色多普勒血流显像甄别假阴性,以降低漏诊率。